United 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
Bentlage ; H ; 605* ; Human diseases with defects in oxidative phosphorylation. 1. Decreased amounts of assembled oxidative phosphorylation complexes in mitochondrial encephalomyopathies. ; 1995 ; 227(3) ; 909-15
Berio ; A ; 3202 hem ; Marinesco-Sjogren syndrome with chronic progressive ophthalmoplegia caused by presumed defective oxidative phosphorylation ; Pediatr Med Chir ; 1996 ; 18(1) ; 99-103
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
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
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
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
Ferrandiz ; ML ; 396* ; Impairment of mitochondrial oxidative phosphorylation in the brain of aged mice. ; Brain Res ; 1994 ; 644(2) ; 335-8
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
Greenbaum ; NL ; 398* ; Role of intramitochondrial pH in the energetics and regulation of mitochondrial oxidative phosphorylation. ; Biochim Biophys Acta ; 1991 ; 1058(2) ; 113-20
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-
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
Issartel ; J.P. ; 177* ; The ATP synthase (F0-F1) complex in oxidative phosphorylation ; Experientia ; 1992 ; 48(4) ; 351-62
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
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
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
Moreno-Sanchez ; R. ; 223* ; Distribution of control of oxidative phosphorylation in mitochondria oxidizing NAD-linked substrates ; Biochim Biophys Acta ; 1991 ; 1060(3) ; 284-92
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
North ; K ; 3878 ; Oxidative phosphorylation defect associated with primary adrenal insufficiency. ; J Pediatr ; 1996 ; 128(5) ; 688-692
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
Rapoport ; SI ; 6180 ; Brain energy metabolism, cognitive function and down-regulated oxidative phosphorylation in Alzheimer disease. ; Neurodegeneration ; 1996 ; 5(4) ; 473-6
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
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
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
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
Senior ; A.E. ; 284* ; ATP Synthesis by Oxidative Phosphorylation ; Physiol Rev ; 1988 ; 68 ; 177-231
Shinozawa ; S. ; 243* ; Effect of Aclarubicin and Doxorubicin on Rat Liver Mitochondrial Oxidative Phosphorylation ; Physiol Chem Phys Med NMR ; 1991 ; 23(2) ; 101-6
Shoffner ; JM ; 109* ; Subacute necrotizing encephalopathy: oxidative phosphorylation defects and the ATPase 6 point mutation. ; Neurology ; 1992 ; 42(11) ; 2168-74
Shoffner ; John M. ; 523* ; Oxidative Phosphorylation Diseases ; The Metabolic Basis of Inherited Desease, 7th edition, Chapter 46 ; 1995 ; 1 ; 1535-1609
Shoffner ; JM ; 4083* ; Maternal inheritance and the evaluation of oxidative phosphorylation diseases. ; Lancet ; 1996 ; 348(9037) ; 1283-1288
Shoffner ; JM ; 1099* ; Renal amino acid transport in adults with oxidative phosphorylation diseases. ; Kidney International ; 1995 ; 47(4) ; 1101-7
Shoffner ; JM ; 1217* ; Oxidative phosphorylation diseases and stroke. [Review] ; Heart Disease & Stroke ; 1993 ; 2(5) ; 439-45
Shoffner ; J.M. ; 44* ; Oxidative Phosphorylation Diseases -- Disorders of Two Genomes ; Advances in Human Genetics ; 1990 ; 19 ; 267-330
Shoffner ; J.M. ; 241* ; Mitochondrial Oxidative Phosphorylation Defects in Parkinson's Disease ; Ann Neurol ; 1991 ; 30(3) ; 332-9
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
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
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
Taylor ; RW ; 1854* ; Defects of oxidative phosphorylation in man. [Review] ; Biochemical Society Transactions ; 1993 ; 21 ( Pt 3)(3) ; 804-7
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
Trounce ; IA ; 4201 ; Assessment of mitochondrial oxidative phosphorylation in patient muscle biopsies, lymphoblasts, and transmitochondrial cell lines. ; Meth Enzymol ; 1996 ; 264 ; 484-509
Wagner ; PD ; 4232* ; Determinants of maximal oxygen transport and utilization. ; Annu Rev Physiol ; 1996 ; 58 ; 21-50
Wallace ; D.C. ; 47* ; Mitochondrial Genes and Disease ; Hospital Practice ; 1986 ; 21 ; 77-92
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
Zerbetto ; E ; 6391 ; Quantification of muscle mitochondrial oxidative phosphorylation enzymes via histochemical staining of blue native polyacrylamide gels. ; Electrophoresis ; 1997 ; 18(11) ; 2059-64