Back to the Subject ListUnited Mitochondrial Disease Foundation
We welcome any suggested additions to our list.
Last updated: 26-Jun-98
REFERENCE FORMAT: Author Lastname; Firstname; Article Number; Article Title; Journal or Book; Year; Volume; Page Numbers
Allen ; RG ; 5677 ; Development and age-associated differences in electron transport potential and consequences for oxidant generation. ; J Biol Chem ; 1997 ; 272(40) ; 24805-12
Ames ; G.F. ; 194* ; Traffic ATPases: A Superfamily of Transport Proteins Operating from Escherichia Coli to Humans ; Adv Enzymol Relat Areas Mol Biol ; 1992 ; 65 ; 1-47
Anderson ; WM ; 2810 ; Cytotoxic effect of thiacarbocyanine dyes on human colon carcinoma cells and inhibition of bovine heart mitochondrial NADH-ubiquinone reductase activity via a rotenone-type mechanism by two of the dyes. ; Biochem Pharmacol ; 1993 ; 45(3) ; 691-6
Ando ; J ; 3129 ; Differential display and cloning of shear stress-responsive messenger RNAs in human endothelial cells. ; Biochem Biophys Res Commun ; 1996 ; 225(2) ; 347-351
Angelini ; C ; 743 ; Hypertrophic cardiomyopathy with mitochondrial myopathy. A new phenotype of complex II defect. ; Japanese Heart Journal ; 1993 ; 34(1) ; 63-77
Antonetti ; DA ; 1377 ; Increased expression of mitochondrial-encoded genes in skeletal muscle of humans with diabetes mellitus. ; J Clinical Investigation ; 1995 ; 95(3) ; 1383-8
Antozzi ; C ; 636* ; Late-onset riboflavin-responsive myopathy with combined multiple acyl coenzyme A dehydrogenase and respiratory chain deficiency. ; Neurology ; 1994 ; 44(11) ; 2153-8
Antozzi ; C ; 3137* ; Epilepsia partialis continua associated with NADH-coenzyme Q reductase deficiency. ; J Neurol Sci ; 1995 ; 129(2) ; 152-161
Bakker ; HD ; 913* ; Vitamin-responsive complex I deficiency in a myopathic patient with increased activity of the terminal respiratory chain and lactic acidosis. ; Journal of Inherited Metabolic Disease ; 1994 ; 17(2) ; 196-204
Barrientos ; A ; 3173 ; A nuclear defect in the 4p16 region predisposes to multiple mitochondrial DNA deletions in families with Wolfram syndrome. ; J Clinical Investigation ; 1996 ; 97(7) ; 1570-1576
Barroso ; N ; 1422* ; Respiratory chain enzyme activities in lymphocytes from untreated patients with Parkinson disease. ; Clinical Chemistry ; 1993 ; 39(4) ; 667-9
Bathori ; G ; 3181* ; Trace amounts of Triton X-100 modify the inhibitor sensitivity of the mitochondrial porin. ; Biochim Biophys Acta ; 1995 ; 1234(2) ; 249-254
Battino ; M ; 1452 ; Structural and functional aspects of the respiratory chain of synaptic and nonsynaptic mitochondria derived from selected brain regions. ; Journal of Bioenergetics & Biomembranes ; 1991 ; 23(2) ; 345-63
Bautista ; J ; 3185 ; Adult onset mitochondrial myopathy without ophthalmoplegia. Four cases attributable to complex III and IV deficits in the respiratory chain ; Neurologia ; 1995 ; 10(8) ; 319-323
Beattie ; DS ; 2306 ; Direct interaction between the internal NADH: ubiquinone oxidoreductase and ubiquinol:cytochrome c oxidoreductase in the reduction of exogenous quinones by yeast mitochondria. ; Archives of Biochemistry & Biophysics ; 1992 ; 292(2) ; 499-505
Beattie ; DS ; 2288 ; A proposed pathway of proton translocation through the bc complexes of mitochondria and chloroplasts. [Review] ; Journal of Bioenergetics & Biomembranes ; 1993 ; 25(3) ; 233-44
Behal ; RH ; 2473* ; Regulation of the pyruvate dehydrogenase multienzyme complex. [Review] ; Annual Review of Nutrition ; 1993 ; 13 ; 497-520
Beharry ; S. ; 143* ; Changes in the adenine nucleotide and inorganic phosphate content of Escherichia coli F1-ATPase during ATP sythesis in dimethyl sulphoxide ; Biochem J. ; 1992 ; 1;286 ; 603-6
Ben-Shachar ; D ; 1393* ; Dopamine neurotoxicity: inhibition of mitochondrial respiration. ; Journal of Neurochemistry ; 1995 ; 64(2) ; 718-23
Benecke ; R ; 2276* ; Electron transfer complexes I and IV of platelets are abnormal in Parkinson's disease but normal in Parkinson-plus syndromes. ; Brain ; 1993 ; 116 ( Pt 6) ; 1451-63
Benito-Leon ; J ; 3192 ; Fibromyalgia in right half of the body as the onset of mitochondrial cytopathy. Letter ; Rev Neurol ; 1996 ; 24(134) ; 1303-1304
Bentlage ; H ; 605* ; Human diseases with defects in oxidative phosphorylation. 1. Decreased amounts of assembled oxidative phosphorylation complexes in mitochondrial encephalomyopathies. ; Eur J Biochem ; 1995 ; 227(3) ; 909-15
Bentlage ; HA ; 3195 ; Immunoprecipitation of human mitochondrial translation products with peptide-specific antibodies. ; Meth Enzymol ; 1996 ; 264 ; 218-228
Bentlage ; HA ; 3196* ; Lethal infantile mitochondrial disease with isolated complex I deficiency in fibroblasts but with combined complex I and IV deficiencies in muscle. ; Neurology ; 1996 ; 47(1) ; 243-248
Bentlage ; HA ; 603* ; Multiple deficiencies of mitochondrial DNA- and nuclear-encoded subunits of respiratory NADH dehydrogenase detected with peptide- and subunit-specific antibodies in mitochondrial myopathies. ; Biochimica et Biophysica Acta ; 1995 ; 1234(1) ; 63-73
Berio ; A ; 3202 ; Marinesco-Sjogren syndrome with chronic progressive ophthalmoplegia caused by presumed defective oxidative phosphorylation ; Pediatr Med Chir ; 1996 ; 18(1) ; 99-103
Bernsen ; PL ; 710* ; Treatment of complex I deficiency with riboflavin. ; Journal of the Neurological Sciences ; 1993 ; 118(2) ; 181-7
Besley ; GT ; 1532* ; Mitochondrial complex deficiencies in a male with cardiomyopathy and 3-methylglutaconic aciduria. ; J Inherit Metab Dis ; 1995 ; 18(2) ; 221-3
Bindoff ; LA ; 2312 ; Multiple defects of the mitochondrial respiratory chain in a mitochondrial encephalopathy (MERRF): a clinical, biochemical and molecular study. [Review] ; Journal of the Neurological Sciences ; 1991 ; 102(1) ; 17-24
Bindoff ; LA ; 2310 ; Respiratory chain abnormalities in skeletal muscle from patients with Parkinson's disease. ; Journal of the Neurological Sciences ; 1991 ; 104(2) ; 203-8
Bindoff ; LA ; 2519 ; Deficiency of the pyruvate dehydrogenase complex and of mitochondrial fatty acid oxidation. [Review] ; Revue Neurologique ; 1991 ; 147(6-7) ; 526-31
Bioulac-Sage ; P ; 847 ; Fatal neonatal liver failure and mitochondrial cytopathy (oxidative phosphorylation deficiency): a light and electron microscopic study of the liver. ; Hepatology ; 1993 ; 18(4) ; 839-46
Birch-Machin ; MA ; 3209* ; Biochemical investigations and immunoblot analyses of two unrelated patients with an isolated deficiency in complex II of the mitochondrial respiratory chain. ; Biochem Biophys Res Commun ; 1996 ; 220(1) ; 57-62
Birch-Machin ; MA ; 2268* ; An evaluation of the measurement of the activities of complexes I-IV in the respiratory chain of human skeletal muscle mitochondria. ; Biochemical Medicine & Metabolic Biology ; 1994 ; 51(1) ; 35-42
Blake ; CI ; 5723 ; Platelet mitochondrial respiratory chain function in Parkinson's disease. ; Mov Disord ; 1997 ; 12(1) ; 3-8
Blandini ; F ; 5724 ; Quantitative study of mitochondrial complex I in platelets of parkinsonian patients. ; Mov Disord ; 1998 ; 13(1) ; 11-5
Blumenthal ; DT ; 5728 ; Myoclonus epilepsy with ragged red fibers and multiple mtDNA deletions. ; Neurology ; 1998 ; 50(2) ; 524-5
Boffoli ; D ; 3215 ; Ageing is associated in females with a decline in the content and activity on the b-c1 complex in skeletal muscle mitochondria. ; Biochim Biophys Acta ; 1996 ; 1315(1) ; 66-72
Boffoli ; D ; 2817* ; Decline with age of the respiratory chain activity in human skeletal muscle. ; Biochim Biophys Acta ; 1994 ; 1226(1) ; 73-82
Boor ; R ; 984 ; Aplasia of the retinal vessels combined with optic nerve hypoplasia, neonatal epileptic seizures, and lactic acidosis due to mitochondrial complex I deficiency. ; European Journal of Pediatrics ; 1992 ; 151(7) ; 519-21
Boumans ; H ; 2240 ; Identification of additional homologues of subunits VII and VIII of the ubiquinol-cytochrome c oxidoreductase enables definition of consensus sequences. ; FEBS Lett ; 1995 ; 368(1) ; 105-9
Boumans ; H ; 3230 ; Topological organization of subunits VII and VIII in the ubiquinol- cytochrome c oxidoreductase of Saccharomyces cerevisiae. ; FEBS Lett ; 1996 ; 390(2) ; 137-141
Bourgeron ; T ; 2885* ; Mutation of a nuclear succinate dehydrogenase gene results in mitochondrial respiratory chain deficiency. ; Nat Genet ; 1995 ; 11(2) ; 144-9
Bourgeron ; T ; 1817 ; Expression of respiratory chain deficiencies in human cultured cells. ; Neuromuscular Disorders ; 1993 ; 3(5-6) ; 605-8
Bourne ; RM ; 1438 ; Characterization of a sodiummotive NADH: ubiquinone oxidoreductase. [Review] ; Biochemical Society Transactions ; 1992 ; 20(3) ; 577-82
Brandt ; U ; 2292* ; The mitochondrial targeting presequence of the Rieske iron-sulfur protein is processed in a single step after insertion into the cytochrome bc1 complex in mammals and retained as a subunit in the complex. ; Journal of Biological Chemistry ; 1993 ; 268(12) ; 8387-90
Brandt ; U ; 3236* ; Energy conservation by bifurcated electron-transfer in the cytochrome- bc1 complex. ; Biochim Biophys Acta ; 1996 ; 1275(1-2) ; 41-46
Brasseur ; G ; 3239 ; Structure-function relationships of the mitochondrial bc1 complex in temperature-sensitive mutants of the cytochrome b gene, impaired in the catalytic center N. ; J Biol Chem ; 1995 ; 270(49) ; 29356-29364
Brasseur ; G ; 3238* ; A compilation of mutations located in the cytochrome b subunit of the bacterial and mitochondrial bc1 complex. ; Biochim Biophys Acta ; 1996 ; 1275(1-2) ; 61-69
Braun ; HP ; 1385* ; Are the 'core' proteins of the mitochondrial bc1 complex evolutionary relics of a processing protease?. ; Trends in Biochemical Sciences ; 1995 ; 20(5) ; 171-5
Braun ; HP ; 3242 ; The bifunctional cytochrome c reductase/processing peptidase complex from plant mitochondria. ; J Bioenergetics & Biomembranes ; 1995 ; 27(4) ; 423-436
Brines ; ML ; 3244* ; Regional distributions of hippocampal Na+,K(+)-ATPase, cytochrome oxidase, and total protein in temporal lobe epilepsy. ; Epilepsia ; 1995 ; 36(4) ; 371-383
Brown ; MD ; 2172 ; Mitochondrial DNA complex I and III mutations associated with Leber's hereditary optic neuropathy. ; Genetics ; 1992 ; 130(1) ; 163-73
Bruel ; C ; 3253* ; Subunit 8 of the Saccharomyces cerevisiae cytochrome bc1 complex interacts with succinate-ubiquinone reductase complex. ; Journal of Bioenergetics & Biomembranes ; 1996 ; 28(1) ; 59-68
Buchwald ; A ; 2707 ; Alterations of the mitochondrial respiratory chain in human dilated cardiomyopathy. ; Eur Heart J ; 1990 ; 11(6) ; 509-16
Burgeois ; M ; 2493 ; Deficiency in complex II of the respiratory chain, presenting as a leukodystrophy in two sisters with Leigh syndrome. ; Brain & Development ; 1992 ; 14(6) ; 404-8
Bussell ; SJ ; 2241* ; Effect of hydrodynamic interactions on the diffusion of integral membrane proteins: tracer diffusion in organelle and reconstituted membranes. ; Biophysical Journal ; 1995 ; 68(5) ; 1828-35
Byrne ; E ; 1952 ; New concepts in respiratory chain diseases. [Review] ; Current Opinion in Rheumatology ; 1992 ; 4(6) ; 784-93
Cain ; B.D. ; 290* ; Proton Translocation by the F1F0ATPase of Escherichia coli: Mutgenic Analysis of the a Subunit ; J Biol Chem ; 1989 ; 264 ; 3292-300
Cain ; B.D. ; 288* ; Impaired Proton Conductivity Resulting from Mutations in the a Subunit of F1F0 ATPase in Escherichia Coli ; J. Biol. Chem. ; 1986 ; 261 ; 10043-50
Cain ; B.D. ; 287* ; Interaction between Glu-219 and His-245 within the a Subunit of F1F0-ATPase in Escherichia coli ; J Biol Chem ; 1988 ; 263 ; 6606-12
Campos ; Y ; 560* ; Respiratory chain enzyme defects in patients with idiopathic inflammatory myopathy. ; Annals of the Rheumatic Diseases ; 1995 ; 54(6) ; 491-3
Campos ; Y ; 5749 ; Leigh syndrome associated with the T9176C mutation in the ATPase 6 gene of mitochondrial DNA. ; Neurology ; 1997 ; 49(2) ; 595-7
Capitanio ; N ; 1450 ; H+/e- stoichiometry of mitochondrial cytochrome complexes reconstituted in liposomes. Rate-dependent changes of the stoichiometry in the cytochrome c oxidase vesicles. ; FEBS Letters ; 1991 ; 288(1-2) ; 179-82
Cardellach ; F ; 395* ; Mitochondrial respiratory chain activity in skeletal muscle from patients with Parkinson's disease [see comments] Department of General Internal Medicine, Hospital Clinic ; Neurology ; 1993 ; 43(11) ; 2258-62
Cassina ; A ; 3276* ; Differential inhibitory action of nitric oxide and peroxynitrite on mitochondrial electron transport. ; Arch Biochem Biophys ; 1996 ; 328(2) ; 309-316
Casta ; A ; 5758 ; Perioperative white matter degeneration and death in a patient with a defect in mitochondrial oxidative phosphorylation. ; Anesthesiology ; 1997 ; 87(2) ; 420-5
Chalmers ; RM ; 3284* ; Sequence of mitochondrial DNA in patients with multiple sclerosis. ; Ann Neurol ; 1996 ; 40(2) ; 239-243
Chomyn ; A. ; 2559 ; Identification of the polypeptides encoded in the ATPase6 gene and in the unassigned reading frames 1 and 3 of human mtDNA. ; Proc. Natl. Acad. Sci. U.S.A. ; 1983 ; 80 ; 5535-5539
Chomyn ; A ; 3296 ; In vivo labeling and analysis of human mitochondrial translation products. ; Meth Enzymol ; 1996 ; 264 ; 197-211
Chretien ; D ; 2687 ; The measurement of the rotenone-sensitive NADH cytochrome c reductase activity in mitochondria isolated from minute amount of human skeletal muscle. ; Biochem Biophys Res Commun ; 1990 ; 173(1) ; 26-33
Chretien ; D ; 1399* ; Reference charts for respiratory chain activities in human tissues. ; Clinica Chimica Acta ; 1994 ; 228(1) ; 53-70
Chun ; K ; 2478 ; Mutations in the X-linked E1 alpha subunit of pyruvate dehydrogenase leading to deficiency of the pyruvate dehydrogenase complex. ; Human Molecular Genetics ; 1993 ; 2(4) ; 449-54
Chun ; K ; 2419* ; Mutations in the X-linked E1 alpha subunit of pyruvate dehydrogenase: exon skipping, insertion of duplicate sequence, and missense mutations leading to the deficiency of the pyruvate dehydrogenase complex [see comments]. ; American Journal of Human Genetics ; 1995 ; 56(3) ; 558-69
Ciulla ; TA ; 3302 ; Bilateral infantile cataractogenesis in a patient with deficiency of complex I, a mitochondrial electron transport chain enzyme. ; J Pediatr Ophthalmol Strabismus ; 1995 ; 32(6) ; 378-382
Collombet ; JM ; 5788 ; Expression of oxidative phosphorylation genes in muscle cell cultures from patients with mitochondrial myopathies. ; Mol Cell Biochem ; 1997 ; 168(1-2) ; 73-85
Cooper ; JM ; 2692 ; Biochemical and molecular aspects of human mitochondrial respiratory chain disorders. ; Biochem Soc Trans ; 1990 ; 18(4) ; 517-9
Cooper ; JM ; 579 ; Treatment of experimental NADH ubiquinone reductase deficiency with menadione. ; Brain ; 1992 ; 115 ( Pt 4) ; 991-1000
Cooper ; JM ; 813 ; Human mitochondrial complex I dysfunction. [Review] ; Biochimica et Biophysica Acta ; 1992 ; 1101(2) ; 198-203
Cornelissen ; J ; 2252* ; Meta-iodobenzylguanidine inhibits complex I and III of the respiratory chain in the human cell line Molt-4. ; Biochemical Pharmacology ; 1995 ; 49(4) ; 471-7
Cornelissen ; JC ; 2078* ; Respiratory chain function in Leber's hereditary optic neuropathy: lack of correlation with clinical disease. ; Journal of Inherited Metabolic Disease ; 1993 ; 16(3) ; 531-3
Cortopassi ; G ; 1091* ; Modelling the effects of age-related mtDNA mutation accumulation; complex I deficiency, superoxide and cell death. ; Biochimica et Biophysica Acta ; 1995 ; 1271(1) ; 171-6
De Jong ; AM ; 1406 ; Ubisemiquinones as obligatory intermediates in the electron transfer from NADH to ubiquinone. ; Eur J Biochem ; 1994 ; 222(3) ; 975-82
De Meirleir ; L ; 5810 ; Pyruvate dehydrogenase complex deficiency and absence of subunit X. ; J Inherit Metab Dis ; 1998 ; 21(1) ; 9-16
de Meis ; L ; 153* ; Reversal of oxidative phosphorylation in submitochondrial particles using glucose 6-phosphate and hexokinase as an ATP regenerating system ; FEBS Lett ; 1992 ; 308(2) ; 197-201
De Vivo ; DC ; 383* ; Defective activation of the pyruvate dehydrogenase complex in subacute necrotizing encephalomyelopathy (Leigh disease) ; Annals of Neurology ; 1979 ; 6 ; 483-94
de Vries ; D. Danielle ; 302* ; A second missense mutation in the mitochondrial ATPase 6 gene in Leigh's syndrome. ; Annals of Neurology ; 1993 ; 34 ; 410-2
De Vries ; DD ; 3345* ; Genetic and biochemical impairment of mitochondrial complex I activity in a family with Leber hereditary optic neuropathy and hereditary spastic dystonia. ; Am J Hum Genet ; 1996 ; 58(4) ; 703-711
De Vries ; S ; 1443 ; Primary structure and import pathway of the rotenone-insensitive NADH-ubiquinone oxidoreductase of mitochondria from Saccharomyces cerevisiae. ; European Journal of Biochemistry ; 1992 ; 203(3) ; 587-92
Degli Esposti ; M ; 2296 ; Complex I and complex III of mitochondria have common inhibitors acting as ubiquinone antagonists. ; Biochemical & Biophysical Research Communications ; 1993 ; 190(3) ; 1090-6
Dimroth ; P ; 159* ; The ATPases of Proionigenium modestum and Bacillus alcalophilus. Strategies for ATP synthesis under low energy conditions ; Biochim Biophys Acta ; 1992 ; 1101(2) ; 236-9
Ding ; H ; 2305 ; Cytochrome bc1 complex [2Fe-2S] cluster and its interaction with ubiquinone and ubihydroquinone at the Qo site: a double-occupancy Qo site model. ; Biochemistry ; 1992 ; 31(12) ; 3144-58
Dionisi-Vici ; C ; 5823 ; Fulminant Leigh syndrome and sudden unexpected death in a family with the T9176C mutation of the mitochondrial ATPase 6 gene. ; J Inherit Metab Dis ; 1998 ; 21(1) ; 2-8
Dionisi-Vici ; C ; 5822 ; New familial mitochondrial encephalopathy with macrocephaly, cardiomyopathy, and complex I deficiency. ; Ann Neurol ; 1997 ; 42(4) ; 661-5
Doring ; O ; 488 ; Effect of D2O on maize plasmalemma ATPase and electron transport coupled proton pumping. ; Biochem Biophys Res Commun ; 1992 ; 182(2) ; 870-6
dos Passos ; J.B. ; 155* ; Glucose-induced activation of plasma membrane H+-ATPase in mutants of the yeast Saccharomyces cerevisiae affected in cAMP metabolism, cAMP-dependent protein phosphorylation and the initiation of glycolysis ; Biochim Biophys Acta ; 1992 ; 1136(1) ; 57-67
Dumoulin ; R ; 5832 ; A novel gly290asp mitochondrial cytochrome b mutation linked to a complex III deficiency in progressive exercise intolerance. ; Mol Cell Probes ; 1996 ; 10(5) ; 389-91
Duncan ; AM ; 2261* ; Assignment of the gene (UQCRFS1) for the Rieske iron-sulfur protein subunit of the mitochondrial cytochrome bc1 complex to the 22q13 and 19q12-q13.1 regions of the human genome. ; Genomics ; 1994 ; 21(1) ; 281-3
Duncan ; AM ; 2277 ; Assignment of the gene for the core protein II (UQCRC2) subunit of the mitochondrial cytochrome bc1 complex to human chromosome 16p12. ; Genomics ; 1993 ; 18(2) ; 455-6
Dunn ; S.D. ; 196* ; The Polar Domain of the b Subunit of Escherichia coli F1F0-ATPase Forms an Elongated Dimer That Interacts with the F1 Sector ; J Biol Chem ; 1992 ; 267(11) ; 7630-6
Engelbrecht ; S. ; 214* ; Subunit d of Chloroplast F0F1-ATPase and OSCP of Mitchondrial F0F1-ATPase: a Comparison by CD-Spectroscopy ; Z Naturforsch [C] ; 1991 ; 46(9-10) ; 759-64
Enriquez ; JA ; 1378* ; MtDNA mutation in MERRF syndrome causes defective aminoacylation of tRNA(Lys) and premature translation termination. ; Nature Genetics ; 1995 ; 10(1) ; 47-55
Ernster ; L ; 2244* ; Biochemical, physiological and medical aspects of ubiquinone function. [Review] ; Biochimica et Biophysica Acta ; 1995 ; 1271(1) ; 195-204
Esposti ; M D ; 2655* ; Complex I and Complex III of Mitochondria Have Common Inhibitors Acting as Ubiquinone Antagonists ; Biochem & Biophys Res Comm ; 1993 ; 190 ; 1090-6
Federico ; A ; 376* ; Congenital lactic acidosis due to a defect of pyruvated dehydrogenase complex (E1). Clinical, biochemical, nerve biopsy study and effect of therapy ; Eur Neurol ; 1990 ; 30(3) ; 123-7
Ferrandiz ; ML ; 396* ; Impairment of mitochondrial oxidative phosphorylation in the brain of aged mice. ; Brain Res ; 1994 ; 644(2) ; 335-8
Figarella-Branger ; D. ; 162* ; Defects of the mitochondrial respiratory chain complexes in three pediatric cases with hypotonia and cardiac involvement ; Journal of the Neurological Sciences ; 1992 ; 108(1) ; 105-13
Finel ; M ; 3415 ; Genetic inactivation of the H(+)-translocating NADH:ubiquinone oxidoreductase of Paracoccus denitrificans is facilitated by insertion of the ndh gene from Escherichia coli. ; FEBS Lett ; 1996 ; 393(1) ; 81-85
Fingerhut ; R ; 1396* ; Impaired degradation of phytanic acid in cells from patients with mitochondriopathies: evidence for the involvement of ETF and the respiratory chain in phytanic acid alpha-oxidation. ; J Inherit Metab Dis ; 1994 ; 17(5) ; 527-32
Finnegan ; MG ; 3416 ; Axial heme ligation in the cytochrome bc1 complexes of mitochondrial and photosynthetic membranes. A near-infrared magnetic circular dichroism and electron paramagnetic resonance study. ; Biochim Biophys Acta ; 1996 ; 1274(1-2) ; 9-20
Flierl ; A ; 5856 ; Pathophysiology of the MELAS 3243 transition mutation. ; J Biol Chem ; 1997 ; 272(43) ; 27189-96
Foldes-Rapp ; Z. ; 151 ; The Animal and Human Plasma Membrane (Ca2+ + Mg2+)-ATPases - Approaches to Molecular Arrangements of Functional Parts and Oxidative Changes ; Gen Physiol Biophys ; 1992 ; 11(1) ; 3-38
Fusi ; F. ; 213* ; The Interaction of Antioxidants and Structurally Related Compounds with Mitochondrial Oxidative Phosphorylation ; Methods Find Exp Clin Pharmacol ; 1991 ; 13(9) ; 599-603
Futai ; M. ; 285* ; ATP SYNTHASE (H+ -ATPase): Results by Combined Biochemical and Molecular Biological Approaches ; Ann Rev Biochem ; 1989 ; 58 ; 111-36
Gardner ; PR ; 2249* ; Superoxide radical and iron modulate aconitase activity in mammalian cells. ; Journal of Biological Chemistry ; 1995 ; 270(22) ; 13399-405
Gavrikova ; EV ; 1383* ; Kinetics of the mitochondrial three-subunit NADH dehydrogenase interaction with hexammineruthenium(III). ; Biochimica et Biophysica Acta ; 1995 ; 1230(1-2) ; 23-30
Geoffroy ; V ; 3446* ; Defect in the X-lipoyl-containing component of the pyruvate dehydrogenase complex in a patient with neonatal lactic acidemia. ; Pediatrics ; 1996 ; 97(2) ; 267-272
Gibson ; KM ; 979 ; 3-Methylglutaconic aciduria associated with Pearson syndrome and respiratory chain defects. ; Journal of Pediatrics ; 1992 ; 121(6) ; 940-2
Glinka ; YY ; 3453 ; Inhibition of mitochondrial complexes I and IV by 6-hydroxydopamine. ; Eur J Pharmacol ; 1995 ; 292(3-4) ; 329-332
Goncalves ; I ; 3456* ; Mitochondrial respiratory chain defect: a new etiology for neonatal cholestasis and early liver insufficiency. ; J Hepatol ; 1995 ; 23(3) ; 290-294
Graham ; LA ; 2266* ; Protease maturation of the Rieske iron-sulphur protein after its insertion into the mitochondrial cytochrome bc1 complex of Saccharomyces cerevisiae. [Review] ; Biochemical Society Transactions ; 1994 ; 22(1) ; 188-91
Graham ; LA ; 2287 ; Mutational analysis of assembly and function of the iron-sulfur protein of the cytochrome bc1 complex in Saccharomyces cerevisiae. [Review] ; Journal of Bioenergetics & Biomembranes ; 1993 ; 25(3) ; 245-57
Gray ; KA ; 1409* ; Requirement of histidine 217 for ubiquinone reductase activity (Qi site) in the cytochrome bc1 complex. ; Biochemistry ; 1994 ; 33(3) ; 723-33
Greenbaum ; NL ; 398* ; Role of intramitochondrial pH in the energetics and regulation of mitochondrial oxidative phosphorylation. ; Biochim Biophys Acta ; 1991 ; 1058(2) ; 113-20
Grunewald ; S ; 5893 ; Neonatal lethal mitochondrial trifunctional protein deficiency mimicking a respiratory chain defect. ; J Inherit Metab Dis ; 1997 ; 20(6) ; 835-6
Gu ; M ; 5895 ; Mitochondrial respiratory chain function in multiple system atrophy. ; Mov Disord ; 1997 ; 12(3) ; 418-22
Gu ; M ; 3074* ; Mitochondrial defect in Huntington's disease caudate nucleus. ; Ann Neurol ; 1996 ; 39(3) ; 385-389
Gudz ; TI ; 5896 ; Direct inhibition of mitochondrial respiratory chain complex III by cell-permeable ceramide. ; J Biol Chem ; 1997 ; 272(39) ; 24154-8
Guedeney ; G ; 3476 ; Evidence for an association of ndh B, ndh J gene products and ferredoxin-NADP-reductase as components of a chloroplastic NAD(P)H dehydrogenase complex. ; FEBS Lett ; 1996 ; 378(3) ; 277-280
Guenthard ; J ; 1615* ; Cardiomyopathy in respiratory chain disorders. [Review] ; Archives of Disease in Childhood ; 1995 ; 72(3) ; 223-6
Haas ; RH ; 857* ; Oxidative metabolism in Rett syndrome: 2. Biochemical and molecular studies. ; Neuropediatrics ; 1995 ; 26(2) ; 95-9
Haas ; RH ; 2247* ; Low platelet mitochondrial complex I and complex II/III activity in early untreated Parkinson's disease. ; Annals of Neurology ; 1995 ; 37(6) ; 714-22
Haferkamp ; O ; 671* ; Mitochondrial complex I and III mutations and neutral-lipid storage in activated mononuclear macrophages and neutrophils: a case presenting with necrotizing myopathy, poikiloderma atrophicans vasculare, and xanthogranulomatous bursitis. ; Human Pathology ; 1994 ; 25(4) ; 419-23
Hansikova ; H ; 2455 ; Deficiency of pyruvate dehydrogenase complex in tissues of an eight month old infant. ; Biochemistry & Molecular Biology International ; 1993 ; 31(6) ; 1157-66
Harada ; M ; 3504* ; Therapeutic efficacy of a case of pyruvate dehydrogenase complex deficiency monitored by localized proton magnetic resonance spectroscopy. ; Magn Reson Imaging ; 1996 ; 14(1) ; 129-133
Harper ; ; 260* ; Oxidative Phosphorylation/Pentose Phosphate Pathway and Gluconeogenesis ; Harper's Biochemistry ; 1988 ; ; 397-445
Hatefi ; Y ; 526 ; The mitochondrial electron transport and oxidative phosphorylation system ; Annu Rev Biochem ; 1985 ; 54 ; 1015-
Hattori ; N ; 2295* ; Immunohistochemical studies on complexes I, II, III, and IV of mitochondria in Parkinson's disease. ; Advances in Neurology ; 1993 ; 60 ; 292-6
Hattori ; N ; 2309 ; Immunohistochemical studies on complexes I, II, III, and IV of mitochondria in Parkinson's disease. ; Annals of Neurology ; 1991 ; 30(4) ; 563-71
Hay ; M ; 3510* ; Construction and characterization of an azurin analog for the purple copper site in cytochrome c oxidase. ; Proc Natl Acad Sci USA ; 1996 ; 93(1) ; 461-464
Heddi ; A ; 1127* ; Steady state levels of mitochondrial and nuclear oxidative phosphorylation transcripts in Kearns-Sayre syndrome. ; Biochimica et Biophysica Acta ; 1994 ; 1226(2) ; 206-12
Hemalatha ; SG ; 2416* ; Pyruvate dehydrogenase complex deficiency due to a point mutation (P188L) within the thiamine pyrophosphate binding loop of the E1 alpha subunit. ; Human Molecular Genetics ; 1995 ; 4(2) ; 315-8
Herzberg ; NH ; 1770* ; Cultured human muscle cells and respiratory chain deficiencies. [Review] ; Neuromuscular Disorders ; 1994 ; 4(1) ; 3-11
Hirai ; M ; 3521 ; Mitochondrial DNA 3394 mutation in the NADH dehydrogenase subunit 1 associated with non-insulin-dependent diabetes mellitus. ; Biochem Biophys Res Commun ; 1996 ; 219(3) ; 951-955
Hirawake ; H ; 2854 ; Human complex II (succinate-ubiquinone oxidoreductase): cDNA cloning of the flavoprotein (Fp) subunit of liver mitochondria. ; J Biochem (Tokyo) ; 1994 ; 116(1) ; 221-7
Hofhaus ; G ; 3527 ; Use of polarography to detect respiration defects in cell cultures. ; Meth Enzymol ; 1996 ; 264 ; 476-483
Hofhaus ; G ; 1425 ; Lack of assembly of mitochondrial DNA-encoded subunits of respiratory NADH dehydrogenase and loss of enzyme activity in a human cell mutant lacking the mitochondrial ND4 gene product [published erratum appears in EMBO J 1994 Dec 1;13(23):5794]. ; EMBO Journal ; 1993 ; 12(8) ; 3043-8
Hofhaus ; G ; 1455 ; Electron microscopic analysis of the peripheral and membrane parts of mitochondrial NADH dehydrogenase (complex I). ; Journal of Molecular Biology ; 1991 ; 221(3) ; 1027-43
Hofhaus ; G ; 3526 ; Respiration and growth defects in transmitochondrial cell lines carrying the 11778 mutation associated with Leber's hereditary optic neuropathy. ; J Biol Chem ; 1996 ; 271(22) ; 13155-13161
Hogue ; DL ; 5926 ; Functional complementation of a membrane transport deficiency in Saccharomyces cerevisiae by recombinant ND4 fusion protein. ; Biochem Biophys Res Commun ; 1997 ; 238(3) ; 811-6
Horisberger ; J.D. ; 244* ; The H, K-ATPase Beta-Subunit Can Act as a Surrogate for the Beta-Subunit of Na, K-pumps ; J Biol Chem ; 1991 ; 266(29) ; 19131-4
Houshmand ; M ; 3537* ; Fatal mitochondrial myopathy, lactic acidosis, and complex I deficiency associated with a heteroplasmic A --> G mutation at position 3251 in the mitochondrial tRNALeu(UUR) gne. ; Human Genetics ; 1996 ; 97(3) ; 269-273
Houstek ; J ; 883* ; Altered properties of mitochondrial ATP-synthase in patients with a T-->G mutation in the ATPase 6 (subunit a) gene at position 8993 of mtDNA. ; Biochimica et Biophysica Acta ; 1995 ; 1271(2-3) ; 349-57
Hu ; G ; 3544 ; Multi-copy nuclear pseudogenes of mitochondrial DNA reveal recent acute genetic changes in the human genome. ; Curr Genet ; 1995 ; 28(5) ; 410-414
Huang ; MY ; 3547 ; Mitochondrial NADH-coenzyme Q reductase deficiency in Leigh's disease. ; J Formos Med Assoc ; 1996 ; 95(4) ; 325-328
Huertas ; R ; 2686 ; Respiratory chain enzymes in muscle of endurance athletes: effect of L-carnitine. ; Biochem Biophys Res Commun ; 1992 ; 188(1) ; 102-7
Ibel ; H ; 931* ; Multiple respiratory chain abnormalities associated with hypertrophic cardiomyopathy and 3-methylglutaconic aciduria. ; European Journal of Pediatrics ; 1993 ; 152(8) ; 665-70
Iglesias ; T ; 1389* ; Identification of the mitochondrial NADH dehydrogenase subunit 3 (ND3) as a thyroid hormone regulated gene by whole genome PCR analysis. ; Biochemical & Biophysical Research Communications ; 1995 ; 210(3) ; 995-1000
Imlay ; JA ; 3563 ; A metabolic enzyme that rapidly produces superoxide, fumarate reductase of Escherichia coli. ; J Biol Chem ; 1995 ; 270(34) ; 19767-19777
Isashiki ; Y ; 2102 ; Sequence homology of NADH CoQ reductase subunit IV with nucleotide-requiring enzymes. ; Japanese Journal of Ophthalmology ; 1993 ; 37(1) ; 39-42
Issartel ; J.P. ; 177* ; The ATP synthase (F0-F1) complex in oxidative phosphorylation ; Experientia ; 1992 ; 48(4) ; 351-62
Jackson ; MJ ; 589* ; Presentation and clinical investigation of mitochondrial respiratory chain disease. A study of 51 patients. ; Brain ; 1995 ; 118 ( Pt 2) ; 339-57
James ; AM ; 3583 ; Altered mitochondrial function in fibroblasts containing MELAS or MERRF mitochondrial DNA mutations. ; Biochem J ; 1996 ; 318( Pt 2) ; 401-407
Janetzky ; B. ; 2576* ; Unaltered aconitase activity, but decreased complex I activity in substantia nigra pars compacta of patients with Parkinson's disease. ; Neurosci. Lett. ; 1994 ; 169 ; 126-128
Japa ; S ; 2248* ; Import of subunit VII of the cytochrome bc1 complex into yeast mitochondrial. ; Archives of Biochemistry & Biophysics ; 1995 ; 319(1) ; 250-6
Johns ; DR ; 1458 ; Allelic mutations of the fourth subunit of NADH dehydrogenase are not pathogenetically important in 11778-negative Leber hereditary optic neuropathy [letter; comment]. ; American Journal of Human Genetics ; 1991 ; 48(6) ; 1209-13
Johns ; DR ; 2196 ; Alternative, simultaneous complex I mitochondrial DNA mutations in Leber's hereditary optic neuropathy. ; Biochemical & Biophysical Research Communications ; 1991 ; 174(3) ; 1324-30
Jordan ; E.M. ; 190* ; Molecular cloning of an import precursor fo the d-subunit of the human mitochondrial ATP synthase complex ; Biochem Biophys Acta ; 1992 ; 1130(1) ; 123-6
Jounouchi ; M. ; 228* ; Role of the Amino Terminal Region of the e Subunit of Escherichia coli H+-ATPase (F0F1) ; Arch Biochem Biophys ; 1992 ; 292(1) ; 87-94
Joynt ; R.J. ; 423* ; Metabolic errors associated with acidosis, Unususal amino acid complexes, and uric acid. Chap 56 ; Inborn Metabolic Errors Affecting the Nervous System, Chap. 56 of Clinical Neurology ; 1992 ; ; 69-71
Jun ; AS ; 1407* ; A mitochondrial DNA mutation at nucleotide pair 14459 of the NADH dehydrogenase subunit 6 gene associated with maternally inherited Leber hereditary optic neuropathy and dystonia. ; Proc Natl Acad Sci USA ; 1994 ; 91(13) ; 6206-10
Jun ; A.S. ; 2581 ; Marked complex I deficiency in patients harboring the np14459 ND6 mitochondrial DNA mutation associated with Leber hereditary optic neuropathy and dystonia. (Abst.1311) ; American Journal of Human Genetics ; 1994 ; 55 (Suppl.) ; A225
Jun ; AS ; 3601 ; Use of transmitochondrial cybrids to assign a complex I defect to the mitochondrial DNA-encoded NADH dehydrogenase subunit 6 gene mutation at nucleotide pair 14459 that causes Leber hereditary optic neuropathy and dystonia. ; Molecular & Cellular Biology ; 1996 ; 16(3) ; 771-777
Kadenbach ; B. ; 227* ; Respiratory Chain Proteins ; Rev Neurol (Paris) ; 1991 ; 147(6-7) ; 436-42
Kalinin ; VN ; 3609 ; A new point mutation in the mitochondrial gene ND1, detected in a patient with type II diabetes ; Genetika ; 1995 ; 31(8) ; 1180-1182
Kataoka ; A ; 5966 ; NADH dehydrogenase deficiency in an apoptosis-resistant mutant isolated from a human HL-60 leukemia cell line. ; Cancer Res ; 1997 ; 57(23) ; 5243-5
Kerr ; DS ; 390* ; Systemic deficiency of the first component of the pyrvate dehydrogenase complex ; Pediatric Research ; 1987 ; 22 ; 312-7
Kerr ; DS ; 5657 ; The purvate dehydrogenase complex and tricarboxylic acid cycle ; in Inborn Metabolic Diseases, edited by Fernandez, et al., 2nd Ed. ; 1995 ; ; 109-20
Kerr ; DS ; 5662 ; Defects of the human pyruvate dehydrogenase complex ; in Alpha Keto Acid Dehydrogenase Complexes, ed: Patel, et al. ; 1996 ; ; 249-69
King ; MP ; 835 ; Defects in mitochondrial protein synthesis and respiratory chain activity segregate with the tRNA(Leu(UUR)) mutation associated with mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes. ; Molecular & Cellular Biology ; 1992 ; 12(2) ; 480-90
Kinoshita ; H ; 5984 ; Recurrent muscle weakness and ataxia in thiamine-responsive pyruvate dehydrogenase complex deficiency. ; J Child Neurol ; 1997 ; 12(2) ; 141-4
Kish ; SJ ; 5985 ; Brain energy metabolizing enzymes in Alzheimer's disease: alpha- ketoglutarate dehydrogenase complex and cytochrome oxidase. ; Ann N Y Acad Sci ; 1997 ; 826 ; 218-28
Kita ; K ; 2688 ; Human complex II (succinate-ubiquinone oxidoreductase): cDNA cloning of iron sulfur (Ip) subunit of liver mitochondria. ; Biochem Biophys Res Commun ; 1990 ; 166(1) ; 101-8
Klohn ; PC ; 3641 ; 2-Nitrosofluorene and N-hydroxy-2-aminofluorene react with the ubiquinone-reduction center (center N) of the mitochondrial cytochrome bc1 complex. ; FEBS Lett ; 1996 ; 389(3) ; 233-237
Klopstock ; T. ; 2586* ; Multiple symmetric lipomatosis: abnormalities in complex IV and multiple deletions in mitochondrial DNA. ; Neurology. ; 1994 ; 44 ; 862-866
Kodama ; S ; 1381 ; Elevated expression of mitochondrial cytochrome b and NADH dehydrogenase subunit 4/4L genes in senescent human cells. ; Experimental Cell Research ; 1995 ; 219(1) ; 82-6
Koga ; Y. ; 89* ; Findings on muscle in complex I (NADH coenzyme Q reductase) deficiency ; Ann Neurol ; 1988 ; 24 ; 749-56
Korzeniewshi ; B. ; 242* ; An extended dynamic model of oxidative phosphorylation ; Biochem Biophys Acta ; 1991 ; 1060(2) ; 210-23
Korzeniewski ; B ; 6002 ; Theoretical studies on control of oxidative phosphorylation in muscle mitochondria at different energy demands and oxygen concentrations. ; Acta Biotheor ; 1996 ; 44(3-4) ; 263-9
Kosel ; S ; 1401* ; No association of mutations at nucleotide 5460 of mitochondrial NADH dehydrogenase with Alzheimer's disease. ; Biochemical & Biophysical Research Communications ; 1994 ; 203(2) ; 745-9
Krahenbuhl ; S ; 3657* ; Determination of the activities of the enzyme complexes of the electron transport chain in human fibroblasts. ; Clin Chim Acta ; 1996 ; 253(1-2) ; 79-90
Kraiczy ; P ; 3658 ; The molecular basis for the natural resistance of the cytochrome bc1 complex from strobilurin-producing basidiomycetes to center Qp inhibitors. ; Eur J Biochem ; 1996 ; 235(1-2) ; 54-63
Kretzschmar ; Hans A. ; 263* ; Pyruvate Dehydrogenase Complex Deficiency as a Cause of Subacute Necrotizing Encephalopathy (Leigh Disease) ; Pediatrics ; 1987 ; 79(3) ; 370-3
Krumholz ; L.R. ; 175* ; Characterization of the genes codinf for the F1F0 subunits of the sodium dependent ATPase of Propionigenium modestum ; FEMS Microbiol Lett ; 1992 ; 70(1) ; 37-41
Kumar ; NR ; 2321* ; Facile and restricted pathways for the dissociation of octenoyl-CoA from the medium-chain fatty acyl-CoA dehydrogenase (MCAD)-FADH2-octenoyl-CoA charge-transfer complex: energetics and mechanism of suppression of the enzyme's oxidase activity. ; Biochemistry ; 1995 ; 34(29) ; 9434-43
Kumar ; S ; 3668 ; Patterns of nucleotide substitution in mitochondrial protein coding genes of vertebrates. ; Genetics ; 1996 ; 143(1) ; 537-548
Kurlemann ; G ; 3673 ; Therapy of complex I deficiency: peripheral neuropathy during dichloroacetate therapy. ; Eur J Pediatr ; 1995 ; 154(11) ; 928-932
Kuznetsov ; AV ; 6008 ; Application of inhibitor titrations for the detection of oxidative phosphorylation defects in saponin-skinned muscle fibers of patients with mitochondrial diseases. ; Biochim Biophys Acta ; 1997 ; 1360(2) ; 142-50
Lamminen ; T ; 2033* ; A mitochondrial mutation at nt 9101 in the ATP synthase 6 gene associated with deficient oxidative phosphorylation in a family with Leber hereditary optic neuroretinopathy [letter]. ; American Journal of Human Genetics ; 1995 ; 56(5) ; 1238-40
Larsson ; NG ; 2185 ; Leber's hereditary optic neuropathy and complex I deficiency in muscle. ; Annals of Neurology ; 1991 ; 30(5) ; 701-8
Laubscher ; B ; 6011 ; Ragged-red fibers and complex I deficiency in a neonate with arthrogryposis congenita. ; Pediatr Neurol ; 1997 ; 17(3) ; 249-51
Lawen ; A ; 1655* ; The universality of bioenergetic disease: the role of mitochondrial mutation and the putative inter-relationship between mitochondria and plasma membrane NADH oxidoreductase. [Review] ; Molecular Aspects of Medicine ; 1994 ; 15 Suppl ; s13-27
Lee ; R.S ; 154* ; F1-ATPase with Cysteine Instead of Serine at Residue 373 of the alpha Subunit ; Arch Biochem Biophys ; 1992 ; 297(2) ; 334-9
Lee ; CC ; 777 ; Chronic progressive external ophthalmoplegia with NADH-CoQ reductase deficiency: report of a case. ; Chung Hua i Hsueh Tsa Chih - Chinese Medical Journal ; 1992 ; 50(1) ; 77-82
Lee ; JW ; 3687* ; Preliminary cryocrystallographic study of the mitochondrial cytochrome bc1 complex: improved crystallization and flash-cooling of a large membrane protein. ; J Mol Biol ; 1995 ; 252(1) ; 15-19
Lefai ; E ; 2253* ; Enzymatic activities of mitochondrial respiratory complexes from children muscular biopsies. Age-related evolutions. ; Biochimica et Biophysica Acta ; 1995 ; 1228(1) ; 43-50
Leone ; TC ; 2322* ; The human medium chain Acyl-CoA dehydrogenase gene promoter consists of a complex arrangement of nuclear receptor response elements and Sp1 binding sites. ; Journal of Biological Chemistry ; 1995 ; 270(27) ; 16308-14
Lertrit ; P ; 966 ; A new disease-related mutation for mitochondrial encephalopathy lactic acidosis and strokelike episodes (MELAS) syndrome affects the ND4 subunit of the respiratory complex I [see comments]. ; American Journal of Human Genetics ; 1992 ; 51(3) ; 457-68
Lestienne ; P. ; 2589 ; Mitochondrial and nuclear DNA complementation in the respiratory chain function and defects. ; Biochimie. ; 1989 ; 71 ; 1115-1123
Lestienne ; P ; 3087 ; Normal mitochondrial genome in brain from patients with Parkinson's disease and complex I defect ; J. Neurochem. ; 1990 ; 55(5) ; 1810-1812
Li ; L ; 2238 ; Effect of propensity of hexagonal II phase formation on the activity of mitochondrial ubiquinol-cytochrome c reductase and H(+)-ATPase. ; Chemistry & Physics of Lipids ; 1995 ; 76(2) ; 135-44
Lin ; FH ; 1431 ; Detection of point mutations in codon 331 of mitochondrial NADH dehydrogenase subunit 2 in Alzheimer's brains. ; Biochemical & Biophysical Research Communications ; 1992 ; 182(1) ; 238-46
Lincke ; CR ; 3705 ; Cerebellar hypoplasia in respiratory chain dysfunction ; Neuropediatrics ; 1996 ; 27(4) ; 216-218
Ling ; M ; 6024 ; Detection of a homozygous four base pair deletion in the protein X gene in a case of pyruvate dehydrogenase complex deficiency. ; Hum Mol Genet ; 1998 ; 7(3) ; 501-5
Lissens ; W ; 3708* ; Mutation analysis of the pyruvate dehydrogenase E1 alpha gene in eight patients with a pyruvate dehydrogenase complex deficiency. ; Human Mutation ; 1996 ; 7(1) ; 46-51
Lorusso ; M ; 2239 ; Proton/electron stoichiometry of mitochondrial bc1 complex. Influence of pH and transmembrane delta pH. ; J Bioenergetics & Biomembranes ; 1995 ; 27(1) ; 101-8
Lotina-Hennsen ; B ; 2251* ; DBHBM (3,5-dibromo-4-hydroxy-benzylidenemalonitrile) is a novel inhibitor of electron transfer through the QN center of the mitochondrial bc1 complex. ; Arch Biochem Biophys ; 1995 ; 318(1) ; 200-6
Lubben ; M ; 1430 ; An archaebacterial terminal oxidase combines core structures of two mitochondrial respiratory complexes. ; EMBO Journal ; 1992 ; 11(3) ; 805-12
Macaulay ; VA ; 6032 ; mtDNA mutation rates--no need to panic [letter; comment] ; Am J Hum Genet ; 1997 ; 61(4) ; 983-90
Majamaa ; K ; 6037 ; Metabolic interventions against complex I deficiency in MELAS syndrome. ; Mol Cell Biochem ; 1997 ; 174(1-2) ; 291-6
Majander ; A ; 1457 ; Electron transfer properties of NADH:ubiquinone reductase in the ND1/3460 and the ND4/11778 mutations of the Leber hereditary optic neuroretinopathy (LHON). ; FEBS Letters ; 1991 ; 292(1-2) ; 289-92
Majander ; A ; 2838* ; Catalytic activity of complex I in cell lines that possess replacement mutations in the ND genes in Leber's hereditary optic neuropathy. ; Eur J Biochem ; 1996 ; 239(1) ; 201-7
Malaney ; S ; 3743 ; Localization of the human gene encoding the 13.3-kDa subunit of mitochondrial complex III (UQCRB) to 8q22 by in situ hybridization. ; Cytogenet Cell Genet ; 1996 ; 73(4) ; 297-299
Marin-Garcia ; J ; 6048 ; A point mutation in the cytb gene of cardiac mtDNA associated with complex III deficiency in ischemic cardiomyopathy. ; Biochem Mol Biol Int ; 1996 ; 40(3) ; 487-95
Marsac ; C ; 2464* ; Defect in the lipoyl-bearing protein X subunit of the pyruvate dehydrogenase complex in two patients with encephalomyelopathy. ; Journal of Pediatrics ; 1993 ; 123(6) ; 915-20
Marusich ; MF ; 6052 ; Expression of mtDNA and nDNA encoded respiratory chain proteins in chemically and genetically-derived Rho0 human fibroblasts: a comparison of subunit proteins in normal fibroblasts treated with ethidium bromide and fibroblasts from a patient ; Biochim Biophys Acta ; 1997 ; 1362(2-3) ; 145-59
Matsumo-Yagi ; A ; 3775* ; Ubiquinol-cytochrome c oxidoreductase. The redox reactions of the bis- heme cytochrome b in ubiquinone-sufficent and ubiquinone-deficient systems. ; J Biol Chem ; 1996 ; 271(11) ; 6164-6171
Maurer ; I ; 6060 ; Inhibition of complex I by neuroleptics in normal human brain cortex parallels the extrapyramidal toxicity of neuroleptics. ; Mol Cell Biochem ; 1997 ; 174(1-2) ; 255-9
Maurer ; I ; 2675 ; Coenzyme Q10 and respiratory chain enzyme activities in hypertrophied human left ventricles with aortic valve stenosis. ; Am J Cardiol ; 1990 ; 66(4) ; 504-5
Maurer ; I ; 2782 ; Myocardial respiratory chain enzyme activities in idiopathic dilated cardiomyopathy, and comparison with those in atherosclerotic coronary artery disease and valvular aortic stenosis. ; Am J Cardiol ; 1993 ; 72(5) ; 428-33
Maurer ; I ; 2262 ; Mitochondrial respiratory chain enzyme activities in tetralogy of Fallot. ; Clinical Investigator ; 1994 ; 72(5) ; 358-63
Maurer ; I ; 2703 ; Positive correlation between aortic valve pressure gradient and mitochondrial respiratory chain capacity in hypertrophied human left ventricle. ; Clin Investig ; 1992 ; 70(10) ; 896-901
Mazzella ; M ; 6063 ; Severe complex I deficiency in a case of neonatal-onset lactic acidosis and fatal liver failure. ; Acta Paediatr ; 1997 ; 86(3) ; 326-9
Meier-Ruge ; WA ; 6069 ; Pathogenesis of decreased glucose turnover and oxidative phosphorylation in ischemic and trauma-induced dementia of the Alzheimer type. ; Ann N Y Acad Sci ; 1997 ; 826 ; 229-41
Mierzewska ; H ; 3800 ; Mitochondrial diseases. Part I -- general review ; Neurol Neurochir Pol ; 1996 ; 30(2) ; 265-278
Mizuno ; Y ; 3088* ; An immunohistochemical study on alpha-ketoglutarate dehydrogenase complex in Parkinson's disease. ; Ann Neurol ; 1994 ; 35(2) ; 204-210
Mizuno ; Y. ; 2597 ; Deficiencies in complex I subunits of the respiratory chain in Parkinson's disease. ; Biochem. Biophys. Res. Commun. ; 1989 ; 163 ; 1450-1455
Molina ; JA ; 6093 ; Respiratory chain enzyme activities in isolated mitochondria of lymphocytes from patients with Alzheimer's disease. ; Neurology ; 1997 ; 48(3) ; 636-8
Monk ; B.C. ; 238* ; Cloning and Characterization of the Plasma Membrane H+-ATPase from Candida albicans ; J Bacteriol ; 1991 ; 173(21) ; 6826-36
Monkami ; A. ; 216* ; The d'-Subunit of Higher Plant Six-subunit Mitochondrial F1-ATPase Is Homologous to the d-Subunit of Animal Mitochondrial F1-ATPase ; J Biol Chem ; 1992 ; 267(1) ; 72-6
Moreno-Sanchez ; R. ; 223* ; Distribution of control of oxidative phosphorylation in mitochondria oxidizing NAD-linked substrates ; Biochim Biophys Acta ; 1991 ; 1060(3) ; 284-92
Morgan-Hughes ; J.A. ; 225* ; The Molecular Pathology of Human Respiratory Chain Defects ; Rev Neurol (Paris) ; 1991 ; 147(6-7) ; 450-4
Morgan-Hughes ; JA ; 1094* ; Mitochondrial DNA (mtDNA) diseases: correlation of genotype to phenotype. ; Biochimica et Biophysica Acta ; 1995 ; 1271(1) ; 135-40
Morris ; AA ; 870* ; Neonatal Fanconi syndrome due to deficiency of complex III of the respiratory chain. ; Pediatric Nephrology ; 1995 ; 9(4) ; 407-11
Morris ; AA ; 1614* ; The investigation of mitochondrial respiratory chain disease. ; Journal of the Royal Society of Medicine ; 1995 ; 88(4) ; 217P-222P
Morris ; AA ; 3819* ; Deficiency of respiratory chain complex I is a common cause of Leigh disease. ; Ann Neurol ; 1996 ; 40(1) ; 25-30
Mourmans ; J ; 6103 ; Clinical heterogeneity in respiratory chain complex III deficiency in childhood. ; J Neurol Sci ; 1997 ; 149(1) ; 111-7
Mousson ; B ; 1526* ; An abnormal exercise test response revealing a respiratory chain complex III deficiency. ; Acta Neurologica Scandinavica ; 1995 ; 91(6) ; 488-93
Muller-Hocker ; J ; 1448 ; Fatal infantile mitochondrial cardiomyopathy and myopathy with heterogeneous tissue expression of combined respiratory chain deficiencies. ; Virchows Archiv - A, Pathological Anatomy & Histopathology ; 1991 ; 419(4) ; 355-62
Muller-Hocker ; J ; 728* ; Generalized mitochondrial microangiopathy and vascular cytochrome c oxidase deficiency. Occurrence in a case of MELAS syndrome with mitochondrial cardiomyopathy-myopathy and combined complex I/IV deficiency. ; Archives of Pathology & Laboratory Medicine ; 1993 ; 117(2) ; 202-10
Muller-Hocker ; J ; 6106 ; Defects of the respiratory chain in the normal human liver and in cirrhosis during aging. ; Hepatology ; 1997 ; 26(3) ; 709-19
Munnich ; Arnold ; 524* ; The Respiratory Chain ; ; 1995 ; ; 1-27
Munnich ; A ; 3839* ; Clinical presentations and laboratory investigations in respiratory chain deficiency. ; Eur J Pediatr ; 1996 ; 155(4) ; 262-274
Nachman ; MW ; 3843 ; Nonneutral mitochondrial DNA variation in humans and chimpanzees. ; Genetics ; 1996 ; 142(3) ; 953-963
Nagai ; T ; 1252 ; Fatal infantile mitochondrial encephalomyopathy with complex I and IV deficiencies. ; Pediatric Neurology ; 1993 ; 9(2) ; 151-4
Nagamune ; H ; 502 ; The lipophilic weak base (Z)-5-methyl-2-[2-(1-naphthyl)ethenyl]-4-piperidinopyridine (AU-1421) is a potent protonophore type cationic uncoupler of oxidative phosphorylation in mitochondria. ; Biochim Biophys Acta ; 1993 ; 1141(2-3) ; 231-7
Nakazono ; M ; 3855 ; A chloroplast-derived sequence is utilized as a source of promoter sequences for the gene for subunit 9 of NADH dehydrogenase (nad9) in rice mitochondria. ; Mol Gen Genet ; 1996 ; 252(4) ; 371-378
Nardelli ; M. ; 2600* ; Detection of novel transcripts in the human mitochondrial DNA region coding for ATPase8-ATPase6 subunits. ; FEBS Lett. ; 1994 ; 344 ; 10-4
Nelson ; N. ; 164* ; Evolution of organellar proton-ATPases ; Biochim Biophys Acta ; 1992 ; 1100(2) ; 109-24
Noguchi ; S. ; 256* ; Assembly of a Hybrid from the alpha Subunit of Na+/K+-ATPase and the beta Subunit of H+/K+-ATPase ; Biochemical and Biophysical Research Communications ; 1992 ; 182(2) ; 659-66
Nohl ; H ; 3875* ; The effect of the exogenous NADH dehydrogenase of heart mitochondria on the transmembranous proton movement. ; Arch Biochem Biophys ; 1996 ; 331(2) ; 259-264
North ; K ; 3878 ; Oxidative phosphorylation defect associated with primary adrenal insufficiency. ; J Pediatr ; 1996 ; 128(5) ; 688-692
Nyhan ; ; 2669 ; Lactic Acidemia--Deficiency of the Pyruvate Dehydrogenase Complex. ; Nyhan textbook, Chap 38 ; ; ; 221-37
O'Donnell ; VB ; 1379* ; Involvement of oxidants and oxidant-generating enzyme(s) in tumour-necrosis-factor-alpha-mediated apoptosis: role for lipoxygenase pathway but not mitochondrial respiratory chain. ; Biochemical Journal ; 1995 ; 310 ( Pt 1) ; 133-41
Obermaier-Kusser ; B ; 1001 ; Respiratory chain activity in tissues from patients (MELAS) with a point mutation of the mitochondrial genome [tRNA(Leu(UUR))]. ; FEBS Letters ; 1991 ; 286(1-2) ; 67-70
Oexle ; K ; 6129 ; Advanced telomere shortening in respiratory chain disorders. ; Hum Mol Genet ; 1997 ; 6(6) ; 905-8
Ogle ; RF ; 6130 ; Mitochondrial myopathy with tRNA(Leu(UUR)) mutation and complex I deficiency responsive to riboflavin. ; J Pediatr ; 1997 ; 130(1) ; 138-45
Oostra ; RJ ; 2006* ; The mitochondrial DNA mutation ND6*14,484C associated with leber hereditary optic neuropathy, leads to deficiency of complex I of the respiratory chain. ; Biochemical & Biophysical Research Communications ; 1995 ; 215(3) ; 1001-5
Papa ; S ; 1394 ; Mechanistic and phenomenological features of proton pumps in the respiratory chain of mitochondria. [Review] ; J Bioenergetics & Biomembranes ; 1994 ; 26(6) ; 609-18
Patel ; MS ; 3928 ; Gene regulation and genetic defects in the pyruvate dehydrogenase complex. [Review] ; J Nutr ; 1995 ; 125(6) ; 1753S-1757S
Patel ; MS ; 3929 ; Mammalian alpha-keto acid dehydrogenase complexes: gene regulation and genetic defects. ; FASEB J ; 1995 ; 9(12) ; 1164-1172
Pearce ; RE ; 3933* ; Effects of freezing, thawing, and storing human liver microsomes on cytochrome P450 activity. ; Arch Biochem Biophys ; 1996 ; 331(2) ; 145-169
Pitkanen ; S ; 3947 ; Mitochondrial complex I deficiency leads to increased production of superoxide radicals and induction of superoxide dismutase. ; J Clinical Investigation ; 1996 ; 98(2) ; 345-351
Pitkanen ; S ; 6163 ; Diagnosis of complex I deficiency in patients with lactic acidemia using skin fibroblast cultures. ; Biochem Mol Med ; 1996 ; 59(2) ; 134-7
Ponitz ; J ; 505 ; A glucose-activated electron transfer system in the plasma membrane stimulates the H(+)-ATPase in Penicillium cyclopium. ; J Bacteriol ; 1994 ; 176(17) ; 5429-38
Priest ; JW ; 3967* ; The trypanosomatid Rieske iron-sulfur proteins have a cleaved presequence that may direct mitochondrial import. ; Biochim Biophys Acta ; 1995 ; 1269(3) ; 201-204
Quentin ; E ; 364* ; Temperature dependence of the coupling efficiency of rat liver oxidative phosphorylation: role of adenine nucleotide translocator ; Biochem Biophys Res Commun ; 1994 ; 202(2) ; 816-21
Radda ; GK ; 1855* ; Control of oxidative phosphorylation in muscle. [Review] ; Biochemical Society Transactions ; 1993 ; 21 ( Pt 3)(3) ; 762-4
Rafferty ; S ; 3973* ; High reductase activity of recombinant NOS2 flavoprotein domain lacking the calmodulin binding regulatory sequence. ; Biochem Biophys Res Commun ; 1996 ; 220(3) ; 1002-1007
Ramana ; KV ; 3976 ; Induction of hepatic cytochrome P450 by phenobarbitone in rhesus monkey (Macaca mulatta). ; Drug Chem Toxicol ; 1996 ; 19(1-2) ; 97-107
Rand ; DM ; 3978 ; Excess amino acid polymorphism in mitochondrial DNA: contrasts among genes from Drosophila, mice, and humans. ; Mol Biol Evol ; 1996 ; 13(6) ; 735-748
Rapoport ; SI ; 6180 ; Brain energy metabolism, cognitive function and down-regulated oxidative phosphorylation in Alzheimer disease. ; Neurodegeneration ; 1996 ; 5(4) ; 473-6
Rasmussen ; O.F. ; 150* ; Nucleotide sequence, organization and characterization of the atp genes and the encoded subunits of Mycoplasma gallisepticum ATPase ; Biochem J ; 1992 ; 285(Pt 3) ; 881-8
Reichmann ; H ; 1520* ; Sarcoid myopathy and mitochondrial respiratory chain defects: clinicopathological, biochemical and molecular biological analyses. ; Neuromuscular Disorders ; 1995 ; 5(4) ; 277-83
Reil ; E ; 1408* ; Synthesis of quinolones and acridones and their inhibitory activity in NADH-dehydrogenases and cytochrome b/c1-complexes. ; Biochemical Society Transactions ; 1994 ; 22(1) ; 62S
Reymann ; S ; 3984 ; Further evidence for multitopological localization of mammalian porin (VDAC) in the plasmalemma forming part of a chloride channel complex affected in cystic fibrosis and encephalomyopathy. ; Biochem Mol Med ; 1995 ; 54(2) ; 75-87
Rich ; PR ; 2298 ; Soret and visible band circular dichroism measurements on the cytochrome bc1 and bf complexes. ; Biochemical Society Transactions ; 1992 ; 20(3) ; 241S
Roberts ; TH ; 3994 ; Direct evidence for the presence of two external NAD(P)H dehydrogenases coupled to the electron transport chain in plant mitochondria. ; FEBS Lett ; 1995 ; 373(3) ; 307-309
Robinson ; BH ; 3995 ; Disorders of pyruvate carboxylase and the pyruvate dehydrogenase complex ; J Inherit Metab Dis ; 1996 ; 19(4) ; 452-462
Robinson ; BH ; 506* ; MtDNA and nuclear mutations affecting oxidative phosphorylation: correlating severity of clinical defect with extent of bioenergetic compromise. ; Journal of Bioenergetics & Biomembranes ; 1994 ; 26(3) ; 311-6
Robinson ; BH ; 388* ; Variable clinical presentaion in patients with deficiency of the pyruvate dehydrogenase complex: a review of 30 cases with a defect in the E1 [pyruvate decarboxylase] component of the complex ; J Pediatr ; 1987 ; 111(4) ; 525-33
Robinson ; B.H. ; 304* ; Four families with variable presentation of the 8993 mtDNA mutation within the ATPase 6 gene ; Am J Hum Genet ; 1992 ; 51 ; A175
Robinson ; B.H. ; 282* ; Respiratory Chain Defects in the Mitochondria of Cultured Skin Fibroblasts from Three Patients with Lacticacidemia ; J Clin Invest ; 1986 ; 77 ; 1422-27
Robinson ; BH ; 2648* ; Clinical presentation of mitochondrial respiratory chain defects in NADH-coenzyme Q reductase and cytochrome oxidase: Clues to pathogenesis of Leigh disease ; J Pediatrics ; 1987 ; 110 ; 216-22
Robinson ; BH ; 2485 ; Fatal combined defects in mitochondrial multienzyme complexes in two siblings. ; European Journal of Pediatrics ; 1992 ; 151(5) ; 347-52
Rohlen ; DA ; 1456 ; Relationship between a subunit of NADH dehydrogenase (complex I) and a protein family including subunits of cytochrome reductase and processing protease of mitochondria. ; FEBS Letters ; 1991 ; 278(1) ; 75-8
Romero ; NB ; 2890 ; Infantile familial cardiomyopathy due to mitochondrial complex I and IV associated deficiency. ; Neuromuscul Disord ; 1993 ; 3(1) ; 31-42
Rubio-Gozalbo ; ME ; 6196 ; Favourable clinical course in an infant with severe deficiency of complex III of the respiratory chain combined with less severe deficiencies of complexes I, II and IV. ; Eur J Pediatr ; 1997 ; 156(12) ; 931-4
Rustin ; P ; 1805 ; The investigation of respiratory chain disorders in heart using endomyocardial biopsies. ; J Inherit Metab Dis ; 1993 ; 16(3) ; 541-4
Rustin ; P ; 1424* ; Investigation of respiratory chain activity in human heart. ; Biochemical Medicine & Metabolic Biology ; 1993 ; 50(1) ; 120-6
Rustin ; P ; 2752 ; Assessment of the mitochondrial respiratory chain [letter] ; Lancet ; 1991 ; 338(8758) ; 60
Sachs ; G. ; 207* ; Mammalian phosphorylating ion-motive ATPases ; Curr Opin Cell Biol ; 1991 ; 3(4) ; 685-94
Sacks ; W ; 2527 ; Inhibition of pyruvate dehydrogenase complex (PDHC) by antipsychotic drugs. ; Biological Psychiatry ; 1991 ; 29(2) ; 176-82
Saijo ; H ; 6200 ; Pyruvate dehydrogenase complex deficiency with multiple minor anomalies. ; Acta Paediatr Jpn ; 1997 ; 39(2) ; 230-2
Saitoh ; I ; 2300 ; Comparison of structure of quinone redox site in the mitochondrial cytochrome-bc1 complex and photosystem II (QB site). ; European Journal of Biochemistry ; 1992 ; 209(1) ; 73-9
Savontaus ; ML ; 2027* ; mtDNA mutations in Leber's hereditary optic neuropathy. [Review] ; Biochimica et Biophysica Acta ; 1995 ; 1271(1) ; 261-3
Schagger ; H ; 4040 ; Electrophoretic separation of multiprotein complexes from blood platelets and cell lines: technique for the analysis of diseases with defects in oxidative phosphorylation ; Electrophoresis ; 1996 ; 17(4) ; 709-714
Schagger ; H ; 4041* ; Human diseases with defects in oxidative phosphorylation. 2. F1F0 ATP- synthase defects in Alzheimer disease revealed by blue native polyacrylamide gel electrophoresis. ; Eur J Biochem ; 1995 ; 227(3) ; 916-921
Schapira ; AH ; 2738 ; Mitochondrial complex I deficiency in Parkinson's disease. ; J Neurochem ; 1990 ; 54(3) ; 823-7
Schapira ; AHV ; 6213 ; Human complex I defects in neurodegenerative diseases ; Biochim Biophys Acta ; 1998 ; 1364(2) ; 261-70
Schatz ; G ; 5653* ; Mitochondria: beyond oxidative phosphorylation ; Biochem Biophys Acta ; 1995 ; 1271 ; 123-6
Scholte ; HR ; 6216 ; Rapid isolation of muscle and heart mitochondria, the lability of oxidative phosphorylation and attempts to stabilize the process in vitro by taurine, carnitine and other compounds. ; Mol Cell Biochem ; 1997 ; 174(1-2) ; 61-6
Scholte ; HR ; 2816* ; Riboflavin-responsive complex I deficiency. ; Biochim Biophys Acta ; 1995 ; 1271(1) ; 75-83
Schuelke ; M ; 6219 ; Cloning of the human mitochondrial 51 kDa subunit (NDUFV1) reveals a 100% antisense homology of its 3'UTR with the 5'UTR of the gamma- interferon inducible protein (IP-30) precursor: is this a link between mitochondrial myopathy and inflammation? ; Biochem Biophys Res Commun ; 1998 ; 245(2) ; 599-606
Selvanayagam ; P ; 4058 ; Detection of mitochondrial genome depletion by a novel cDNA in renal cell carcinoma. ; Lab Invest ; 1996 ; 74(3) ; 592-599
Senior ; A.E. ; 152* ; Catalytic Properties of Escherichia coli F1-ATPase Depleted of Endogenous Nucleotides ; Arch Biochem Biophys ; 1992 ; 297(2) ; 340-4
Senior ; A.E. ; 284* ; ATP Synthesis by Oxidative Phosphorylation ; Physiol Rev ; 1988 ; 68 ; 177-231
Service ; RF ; 4063* ; Crystallographers pinpoint what goes where ; Science ; 1996 ; 273(5279) ; 1174-1175
Sharma ; P ; 6231 ; Consequences of aging on mitochondrial respiratory chain enzymes in cultured human fibroblasts treated with ascorbate. ; Gerontology ; 1998 ; 44(2) ; 78-84
Shergill ; JK ; 4071* ; Detection of nitrosyl complexes in human substantia nigra, in relation to Parkinson's disease. ; Biochem Biophys Res Commun ; 1996 ; 228(2) ; 298-305
Shinozawa ; S. ; 243* ; Effect of Aclarubicin and Doxorubicin on Rat Liver Mitochondrial Oxidative Phosphorylation ; Physiol Chem Phys Med NMR ; 1991 ; 23(2) ; 101-6
Shirabe ; K ; 5622* ; A novel point mutation in a 3' splice site of the NADH-cytochrome b5 reductase gene results in immunologically undetectable enzyme and impaired NADH-dependent ascorbate regeneration in cultured fibroblasts of a patient with type II hereditary ; Am J Hum Genet ; 1995 ; 57(2) ; 302-310
Shoffner ; JM ; 109* ; Subacute necrotizing encephalopathy: oxidative phosphorylation defects and the ATPase 6 point mutation. ; Neurology ; 1992 ; 42(11) ; 2168-74
Shoffner ; J.M. ; 241* ; Mitochondrial Oxidative Phosphorylation Defects in Parkinson's Disease ; Ann Neurol ; 1991 ; 30(3) ; 332-9
Shoffner ; JM ; 1217* ; Oxidative phosphorylation diseases and stroke. [Review] ; Heart Disease & Stroke ; 1993 ; 2(5) ; 439-45
Shoffner ; JM ; 1099* ; Renal amino acid transport in adults with oxidative phosphorylation diseases. ; Kidney International ; 1995 ; 47(4) ; 1101-7
Shoffner ; JM ; 4083* ; Maternal inheritance and the evaluation of oxidative phosphorylation diseases. ; Lancet ; 1996 ; 348(9037) ; 1283-1288
Shoffner ; John M. ; 523* ; Oxidative Phosphorylation Diseases ; The Metabolic Basis of Inherited Desease, 7th edition, Chapter 46 ; 1995 ; 1 ; 1535-1609
Shoffner ; J.M. ; 44* ; Oxidative Phosphorylation Diseases -- Disorders of Two Genomes ; Advances in Human Genetics ; 1990 ; 19 ; 267-330
Shults ; CW ; 2254* ; Carbidopa/levodopa and selegiline do not affect platelet mitochondrial function in early parkinsonism. ; Neurology ; 1995 ; 45(2) ; 344-8
Shults ; CW ; 6241 ; Coenzyme Q10 levels correlate with the activities of complexes I and II/III in mitochondria from parkinsonian and nonparkinsonian subjects. ; Ann Neurol ; 1997 ; 42(2) ; 261-4
Singer ; TP ; 1402* ; The reaction sites of rotenone and ubiquinone with mitochondrial NADH dehydrogenase. ; Biochimica et Biophysica Acta ; 1994 ; 1187(2) ; 198-202
Singer ; TP ; 1386* ; Deficiencies of NADH and succinate dehydrogenases in degenerative diseases and myopathies. [Review] ; Biochimica et Biophysica Acta ; 1995 ; 1271(1) ; 211-9
Singh ; PJ ; 1388* ; Quick preparation of mitochondrial DNA fractions free from nuclear DNA for polymerase chain reaction amplification. ; Analytical Biochemistry ; 1995 ; 225(1) ; 155-7
Sled ; VD ; 1419* ; Reductive inactivation of the mitochondrial three subunit NADH dehydrogenase. ; Biochimica et Biophysica Acta ; 1993 ; 1143(2) ; 199-203
Slipetz ; DM ; 2676 ; Deficiency of complex III of the mitochondrial respiratory chain in a patient with facioscapulohumeral disease. ; Am J Hum Genet ; 1991 ; 48(3) ; 502-10
Slipetz ; DM ; 2677 ; Congenital deficiency of a 20-kDa subunit of mitochondrial complex I in fibroblasts. ; Am J Hum Genet ; 1991 ; 48(6) ; 1121-6
Sokol ; RJ ; 4105 ; Abnormalities of esterase and glycogen in developing macrophages in non- Hodgkin's lymphoma: a quantitative cytochemical study. ; Anal Cell Pathol ; 1995 ; 8(4) ; 297-306
Song ; X ; 6258 ; Cytotoxic effects of MPTP on SH-SY5Y human neuroblastoma cells. ; Neurotoxicology ; 1997 ; 18(2) ; 341-53
Soole ; KL ; 4109 ; Functional molecular aspects of the NADH dehydrogenases of plant mitochondria. ; J Bioenergetics & Biomembranes ; 1995 ; 27(4) ; 397-406
Sperl ; W ; 818 ; Combined deficiencies of the pyruvate dehydrogenase complex and enzymes of the respiratory chain in mitochondrial myopathies. ; European Journal of Pediatrics ; 1992 ; 151(3) ; 192-5
Sprecher ; H ; 4114* ; Alteration of mitochondrial gene expression and disruption of respiratory function by the lipophilic antifolate pyrimethamine in mammalian cells. ; J Biol Chem ; 1995 ; 270(35) ; 20668-20676
Sreeramulu ; K ; 510 ; Inhibition of mitochondrial oxidative phosphorylation and its electron transport pathway by a polycation in vitro. ; Indian J Biochem Biophys ; 1993 ; 30(1) ; 49-53
Stein ; Wilfred D. ; 117* ; Chapter 6: Primary Active Transport Systems: Chemiosmosis: Na+,K+ ATPase/Other ATPases with Isolatable Phosphoenzyme Forms/F1F0-ATPases ; Transport and Diffusion across Cell Membranes ; 1986 ; ; 475-612
Suzuki ; H ; 2314 ; Existence of common homologous elements in the transcriptional regulatory regions of human nuclear genes and mitochondrial gene for the oxidative phosphorylation system. ; Journal of Biological Chemistry ; 1991 ; 266(4) ; 2333-8
Swerdlow ; RH ; 3105* ; Origin and functional consequences of the complex I defect in Parkinson's disease. ; Ann Neurol ; 1996 ; 40(4) ; 663-671
Szczepaniak ; A ; 2237* ; Membrane association of the Rieske iron-sulfur protein. ; Zeitschrift fur Naturforschung Section C Journal of Biosciences ; 1995 ; 50(7-8) ; 535-42
Szczesna-Kaczmarek ; A ; 1442 ; Regulating effect of mitochondrial lactate dehydrogenase on oxidation of cytoplasmic NADH via an "external" pathway in skeletal muscle mitochondria. ; International Journal of Biochemistry ; 1992 ; 24(4) ; 657-61
Tanaka ; M ; 407* ; Tissue- and substrate-dependent responses of oxidative phosphorylation to dietary protein level in chicks. ; Br J Nutr ; 1993 ; 70(2) ; 459-69
Tarasiuk ; J ; 1437 ; The essential role of anthraquinones as substrates for NADH dehydrogenase in their redox cycling activity. ; Anti-Cancer Drug Design ; 1992 ; 7(4) ; 329-40
Taylor ; RW ; 4162* ; Deficiency of complex II of the mitochondrial respiratory chain in late- onset optic atrophy and ataxia. ; Ann Neurol ; 1996 ; 39(2) ; 224-232
Taylor ; RW ; 1854* ; Defects of oxidative phosphorylation in man. [Review] ; Biochemical Society Transactions ; 1993 ; 21 ( Pt 3)(3) ; 804-7
Taylor ; RW ; 1790* ; The control of mitochondrial oxidations by complex III in rat muscle and liver mitochondria. Implications for our understanding of mitochondrial cytopathies in man. ; Journal of Biological Chemistry ; 1994 ; 269(5) ; 3523-8
Tengan ; CH ; 6313 ; Oxidative phosphorylation dysfunction does not increase the rate of accumulation of age-related mtDNA deletions in skeletal muscle. ; Mutat Res ; 1997 ; 379(1) ; 1-11
Thyagarajan ; D ; 4176* ; A novel mitochondrial ATPase 6 point mutation in familial bilateral striatal necrosis. ; Ann Neurol ; 1995 ; 38(3) ; 468-472
Tiranti ; V ; 6324 ; A novel mutation in the mitochondrial tRNA(Val) gene associated with a complex neurological presentation. ; Ann Neurol ; 1998 ; 43(1) ; 98-101
Toline ; CA ; 4183 ; Mitochondrial DNA variation and population genetic structure of the northern redbelly dace (Phoxinus eos). ; Mol Ecol ; 1995 ; 4(6) ; 745-753
Tolkatchev ; D ; 4184* ; Potential induced redox reactions in mitochondrial and bacterial cytochrome b-c1 complexes. ; J Biol Chem ; 1996 ; 271(21) ; 12356-12363
Toscano ; A ; 844 ; Early-onset cerebellar ataxia, myoclonus and hypogonadism in a case of mitochondrial complex III deficiency treated with vitamins K3 and C. ; J Neurology ; 1995 ; 242(4) ; 203-9
Trijbels ; JM ; 4199 ; Benign mitochondrial encephalomyopathy in a patient with complex I deficiency. ; J Inherit Metab Dis ; 1996 ; 19(2) ; 149-152
Trijbels ; FJ ; 6327 ; Defects in the mitochondrial energy metabolism outside the respiratory chain and the pyruvate dehydrogenase complex. ; Mol Cell Biochem ; 1997 ; 174(1-2) ; 243-7
Tron ; T ; 2250* ; QSR1, an essential yeast gene with a genetic relationship to a subunit of the mitochondrial cytochrome bc1 complex, is homologous to a gene implicated in eukaryotic cell differentiation. ; Journal of Biological Chemistry ; 1995 ; 270(17) ; 9961-70
Trounce ; Ian ; 261* ; Functional respiratory chain studies in subjects with chronic progressive external ophthalmoplegia and large heteroplasmic mitochondrial DNA deletions ; Journal of the Neurological Sciences ; 1991 ; 102(1) ; 92-9
Trounce ; I. ; 2634 ; Decline in skeletal muscle mitochondrial respiratory chain function: possible factor in ageing. ; Lancet ; 1989 ; 1 ; 637-639
Trounce ; IA ; 4201 ; Assessment of mitochondrial oxidative phosphorylation in patient muscle biopsies, lymphoblasts, and transmitochondrial cell lines. ; Meth Enzymol ; 1996 ; 264 ; 484-509
Trounce ; I ; 854 ; Affinity chromatography isolation of human cytochrome oxidase and small-scale Western immunoblot probing of the enzyme complex in mitochondrial cytopathy patients. ; Biochemical Medicine & Metabolic Biology ; 1991 ; 46(1) ; 17-27
Tsao ; CY ; 1665* ; Partial NADH dehydrogenase defect presenting as spastic cerebral palsy. ; Brain & Development ; 1994 ; 16(5) ; 393-5
Tzagoloff ; A ; 512 ; A new member of a family of ATPases is essential for assembly of mitochondrial respiratory chain and ATP synthetase complexes in Saccharomyces cerevisiae. ; J Biol Chem ; 1994 ; 269(42) ; 26144-51
Uslu ; R ; 2824* ; Involvement of the mitochondrion respiratory chain in the synergy achieved by treatment of human ovarian carcinoma cell lines with both tumor necrosis factor-alpha and cis-diamminedichloroplatinum. ; Cancer ; 1996 ; 77(4) ; 725-32
Uziel ; G ; 6337 ; Mitochondrial disease associated with the T8993G mutation of the mitochondrial ATPase 6 gene: a clinical, biochemical, and molecular study in six families. ; J Neurol Neurosurg Psychiatry ; 1997 ; 63(1) ; 16-22
Vallance ; HD ; 2429* ; Measurement of pyruvate dehydrogenase complex (PDHC) in interleukin-2 (IL-2) stimulated lymphocytes. ; J Inherit Metab Dis ; 1994 ; 17(5) ; 627-8
van Erven ; Paul M.M. ; 258* ; Mitochondrail Encephalomyopathy: Association With an NADH Dehydrogenase Deficiency ; Arch Neurol ; 1987 ; 44 ; 775-8
Vancutsem ; PM ; 4215 ; In vitro and in vivo study of the effects of enrofloxacin on hepatic cytochrome P-450. Potential for drug interactions. ; Vet Hum Toxicol ; 1996 ; 38(4) ; 254-259
Verdu ; A ; 4220* ; Respiratory chain complex I deficiency in an infant with infantile spasms ; J Neurol Neurosurg Psychiatr ; 1996 ; 60(3) ; 349
Vergani ; L ; 1390* ; MtDNA mutations associated with Leber's hereditary optic neuropathy: studies on cytoplasmic hybrid (cybrid) cells. ; Biochemical & Biophysical Research Communications ; 1995 ; 210(3) ; 880-8
von Dobeln ; U ; 939* ; Fatal neonatal lactic acidosis with respiratory insufficiency due to complex I and IV deficiency. ; Acta Paediatrica ; 1993 ; 82(12) ; 1079-81
Vyas ; JM ; 4229* ; H-2M3a violates the paradigm for major histocompatibility complex class I peptide binding. ; J Exp Med ; 1995 ; 181(5) ; 1817-1825
Walker ; DJ ; 4234 ; Mitochondrial NADH-->NAD transhydrogenation in adult Hymenolepis diminuta. ; J Parasitol ; 1995 ; 81(3) ; 350-353
Walker ; UA ; 4235* ; Respiratory chain encephalomyopathies: a diagnostic classification. ; Eur Neurol ; 1996 ; 36(5) ; 260-267
Walker ; UA ; 4236* ; The therapy of respiratory chain encephalomyopathy: a critical review of the past and current perspective. ; Acta Neurol Scand ; 1995 ; 92(4) ; 273-280
Wanders ; RJ ; 1271* ; Studies on mitochondrial oxidative phosphorylation in permeabilized human skin fibroblasts: application to mitochondrial encephalomyopathies. ; Biochimica et Biophysica Acta ; 1993 ; 1181(3) ; 219-22
Wanders ; RJ ; 1493 ; Prenatal diagnosis of systemic disorders of the respiratory chain in cultured amniocytes and chorionic villus fibroblasts by studying the formation of lactate and pyruvate from glucose. ; Journal of Inherited Metabolic Disease ; 1992 ; 15(1) ; 84-91
Weiss ; H ; 1459 ; The respiratory-chain NADH dehydrogenase (complex I) of mitochondria. [Review] ; European Journal of Biochemistry ; 1991 ; 197(3) ; 563-76
Welter ; R ; 4244* ; The effects of nitric oxide on electron transport complexes. ; Arch Biochem Biophys ; 1996 ; 331(1) ; 9-14
Wendel ; U ; 4245 ; Neonatal De Toni-Debre-Fanconi syndrome due to a defect in complex III of the respiratory chain. ; Eur J Pediatr ; 1995 ; 154(11) ; 915-918
Wexler ; ID ; 2501 ; A mutation in the E1 alpha subunit of pyruvate dehydrogenase associated with variable expression of pyruvate dehydrogenase complex deficiency. ; Pediatric Research ; 1992 ; 32(2) ; 169-74
Wijburg ; FA ; 361* ; Leigh syndrome associated with a deficiency of the pyruvate dehydrogenase complex: results of treatment with a ketogenic diet. ; Neuropediatrics ; 1992 ; 23(3) ; 147-52
Wijburg ; FA ; 2735 ; Restoration of NADH-oxidation in complex I and complex III deficient fibroblasts by menadione. ; J Inherit Metab Dis ; 1991 ; 14(3) ; 293-6
Wijburg ; F.A. ; 217* ; NADH:Q1 oxidoreductase deficiency without lactic acidosis in a patient with Leigh syndrome: implications for the diagnosis of inborn errors of the respiratory chain. ; Journal of Inherited Metabolic Disease ; 1991 ; 14(3) ; 297-300
Wikstrom ; M. ; 530 ; The semiquinone cycle. A hypotheis of electron transfer and proton translocation in cytochrome bc-type complexes ; J Bioenerg Biomembr ; 1986 ; 18 ; 181-
Winberg ; LD ; 1392 ; Mechanism of phthalate-induced inhibition of hepatic mitochondrial beta-oxidation. ; Toxicology Letters ; 1995 ; 76(1) ; 63-9
Wise ; CA ; 6375 ; Departure from neutrality at the mitochondrial NADH dehydrogenase subunit 2 gene in humans, but not in chimpanzees. ; Genetics ; 1998 ; 148(1) ; 409-21
Yamada ; EW ; 2693 ; Distribution of the ATPase inhibitor proteins of mitochondria in mammalian tissues including fibroblasts from a patient with Luft's disease. ; Biochim Biophys Acta ; 1992 ; 1139(1-2) ; 143-7
Yang ; M ; 2271* ; Deletion of QCR6, the gene encoding subunit six of the mitochondrial cytochrome bc1 complex, blocks maturation of cytochrome c1, and causes temperature-sensitive petite growth in Saccharomyces cerevisiae. ; Journal of Biological Chemistry ; 1994 ; 269(2) ; 1270-5
Yen ; MY ; 2820 ; Compensatory elevation of complex II activity in Leber's hereditary optic neuropathy. ; Br J Ophthalmol ; 1996 ; 80(1) ; 78-81
Yoshino ; H ; 1432 ; Mitochondrial complex I and II activities of lymphocytes and platelets in Parkinson's disease. ; Journal of Neural Transmission - Parkinsons Disease & Dementia Section ; 1992 ; 4(1) ; 27-34
Yu ; CA ; 4284* ; Crystallization and preliminary structure of beef heart mitochondrial cytochrome-bc1 complex. ; Biochim Biophys Acta ; 1996 ; 1275(1-2) ; 47-53
Yu ; J ; 2232 ; The cytochrome bc complex (menaquinone:cytochrome c reductase) in Bacillus subtilis has a nontraditional subunit organization. ; J Bacteriology ; 1995 ; 177(23) ; 6751-60
Yu ; CA ; 2286 ; Mitochondrial ubiquinol-cytochrome c reductase complex: crystallization and protein: ubiquinone interaction. [Review] ; Journal of Bioenergetics & Biomembranes ; 1993 ; 25(3) ; 259-73
Yu ; L ; 2231* ; Cloning, gene sequencing, and expression of the small molecular mass ubiquinone-binding protein of mitochondrial ubiquinol-cytochrome c reductase. ; Journal of Biological Chemistry ; 1995 ; 270(43) ; 25634-8
Zafeiriou ; DI ; 1462* ; Deficiency in complex IV (cytochrome c oxidase) of the respiratory chain, presenting as a leukodystrophy in two siblings with Leigh syndrome. ; Brain Dev ; 1995 ; 17(2) ; 117-21
Zerbetto ; E ; 6391 ; Quantification of muscle mitochondrial oxidative phosphorylation enzymes via histochemical staining of blue native polyacrylamide gels. ; Electrophoresis ; 1997 ; 18(11) ; 2059-64
Zhang ; LH ; 6397 ; Mitochondrial genomic markers confirm the presence of the camel strain (G6 genotype) of Echinococcus granulosus in north-western China. ; Parasitology ; 1998 ; 116 ( Pt 1) ; 29-33
Zucchini ; C ; 2805* ; Human skeletal muscle mitochondria in aging: lack of detectable morphological and enzymic defects. ; Biochem Mol Biol Int ; 1995 ; 37(4) ; 607-16