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Last updated: 26-Jun-98
REFERENCE FORMAT: Author Lastname; Firstname; Article Number; Article Title; Journal or Book; Year; Volume; Page Numbers
Andresen ; BS ; 3132 ; Cloning and characterization of human very-long-chain acyl-CoA dehydrogenase cDNA, chromosomal assignment of the gene and identification in four patients of nine different mutations within the VLCAD gene. ; 1996 ; 5(4) ; 461-472
Andresen ; BS ; 3135 cular Genetics ; The mutational spectrum in very long-chain acyl-CoA dehydrogenase deficiency. ; J Inherit Metab Dis ; 1996 ; 19(2) ; 169-172
Aoyama ; T ; 5684 ; Assignment of the human mitochondrial very-long-chain acyl-CoA dehydrogenase gene (LCACD) to 17p13 by in situ hybridization. ; Genomics ; 1996 ; 37(1) ; 144-5
Aoyama ; T ; 3138* ; Cloning of human very-long-chain acyl-coenzyme A dehydrogenase and molecular characterization of its deficiency in two patients. ; Am J Hum Genet ; 1995 ; 57(2) ; 273-283
Aoyama ; T ; 3139 ; Purification of human very-long-chain acyl-coenzyme A dehydrogenase and characterization of its deficiency in seven patients. ; J Clinical Investigation ; 1995 ; 95(6) ; 2465-2473
Battaile ; KP ; 5708 ; Human long chain, very long chain and medium chain acyl-CoA dehydrogenases are specific for the S-enantiomer of 2- methylpentadecanoyl-CoA. ; Biochim Biophys Acta ; 1998 ; 1390(3) ; 333-8
Battaile ; KP ; 5709 ; Functional role of the active site glutamate-368 in rat short chain acyl-CoA dehydrogenase. ; Biochemistry ; 1996 ; 35(48) ; 15356-63
Brivet ; M ; 3247* ; Rapid diagnosis of long chain and medium chain fatty acid oxidation disorders using lymphocytes. ; Ann Clin Biochem ; 1995 ; 32( Pt 2) ; 154-159
Coates ; PM ; 2334* ; New developments in the diagnosis and investigation of mitochondrial fatty acid oxidation disorders. [Review] ; European Journal of Pediatrics ; 1994 ; 153(7 Suppl 1) ; S49-56
Coates ; PM ; 3307* ; Very-long-chain acyl-CoA dehydrogenase deficiency: molecular genetics of a mitochondrial membrane enzyme ; Am J Hum Genet ; 1995 ; 57(2) ; 233-234
Costa ; CG ; 5797 ; Quantitative analysis of plasma acylcarnitines using gas chromatography chemical ionization mass fragmentography. ; J Lipid Res ; 1997 ; 38(1) ; 173-82
Costa ; CG ; 3318 ; Organic acid profiles resembling a beta-oxidation defect in two patients with coeliac disease. ; J Inherit Metab Dis ; 1996 ; 19(2) ; 177-180
Eder ; M ; 5835 ; Characterization of human and pig kidney long-chain-acyl-CoA dehydrogenases and their role in beta-oxidation. ; Eur J Biochem ; 1997 ; 245(3) ; 600-7
Kenealy ; S ; 5973 ; The very-long-chain acyl-coA dehydrogenase gene maps to pig chromosome 12. ; J Anim Sci ; 1998 ; 76(3) ; 915-6
Kieweg ; V ; 5976 ; Biochemical characterization of purified, human recombinant Lys304-- >Glu medium-chain acyl-CoA dehydrogenase containing the common disease- causing mutation and comparison with the normal enzyme. ; Eur J Biochem ; 1997 ; 246(2) ; 548-56
Merinero ; B ; 3794 ; Mitochondrial very long-chain acyl-CoA dehydrogenase deficiency with a mild clinical course. ; J Inherit Metab Dis ; 1996 ; 19(2) ; 173-176
Nada ; MA ; 3845* ; Prenatal diagnosis of mitochondrial fatty acid oxidation defects. ; Prenat Diagn ; 1996 ; 16(2) ; 117-124
Nandy ; A ; 3858 ; Molecular evolution and substrate specificity of acyl-CoA dehydrogenases: chimaeric "medium/long' chain-specific enzyme from medium-chain acyl-CoA dehydrogenase. ; Biochem Soc Trans ; 1996 ; 24(1) ; 105-110
Onkenhout ; W ; 2316* ; Identification and quantification of intermediates of unsaturated fatty acid metabolism in plasma of patients with fatty acid oxidation disorders. ; Clinical Chemistry ; 1995 ; 41(10) ; 1467-74
Orii ; KO ; 3903* ; Genomic DNA organization of human mitochondrial very-long-chain acyl- CoA dehydrogenase and mutation analysis. ; Biochem Biophys Res Commun ; 1995 ; 217(3) ; 987-992
Sluysmans ; T ; 6251 ; Very long chain acyl-coenzyme A dehydrogenase deficiency in two siblings: evolution after prenatal diagnosis and prompt management. ; J Pediatr ; 1997 ; 131(3) ; 444-6
Smelt ; AH ; 6252 ; Very long chain acyl-coenzyme A dehydrogenase deficiency with adult onset. ; Ann Neurol ; 1998 ; 43(4) ; 540-4
Souri ; M ; 6260 ; Very-long-chain acyl-CoA dehydrogenase subunit assembles to the dimer form on mitochondrial inner membrane. ; FEBS Lett ; 1998 ; 426(2) ; 187-90
Souri ; M ; 6261 ; Catalytic and FAD-binding residues of mitochondrial very long chain acyl-coenzyme A dehydrogenase. ; J Biol Chem ; 1998 ; 273(7) ; 4227-31
Souri ; M ; 4111* ; Mutation analysis of very-long-chain acyl-coenzyme A dehydrogenase (VLCAD) deficiency: identification and characterization of mutant VLCAD cDNAs from four patients. ; Am J Hum Genet ; 1996 ; 58(1) ; 97-106
Spector ; AA ; 6262 ; Conversion of arachidonic acid to tetradecadienoic acid by peroxisomal oxidation. ; Prostaglandins Leukot Essent Fatty Acids ; 1997 ; 57(1) ; 101-5
Strauss ; AW ; 4123* ; Molecular basis of human mitochondrial very-long-chain acyl-CoA dehydrogenase deficiency causing cardiomyopathy and sudden death in childhood. ; Proc Natl Acad Sci USA ; 1995 ; 92(23) ; 10496-10500
Straussberg ; R ; 6277 ; Recurrent myoglobinuria as a presenting manifestation of very long chain acyl coenzyme A dehydrogenase deficiency. ; Pediatrics ; 1997 ; 99(6) ; 894-6
Tamvakopoulos ; CS ; 4155 ; Long-chain acyl-CoA profiles in cultured fibroblasts from patients with defects in fatty acid oxidation. ; Biochem Mol Med ; 1995 ; 55(1) ; 15-21
Tiffany ; KA ; 6323 ; Structure of human isovaleryl-CoA dehydrogenase at 2.6 A resolution: structural basis for substrate specificity,. ; Biochemistry ; 1997 ; 36(28) ; 8455-64
Vianey-Saban ; C ; 6348 ; Mitochondrial very-long-chain acyl-coenzyme A dehydrogenase deficiency: clinical characteristics and diagnostic considerations in 30 patients. ; Clin Chim Acta ; 1998 ; 269(1) ; 43-62
Williard ; DE ; 6374 ; Conversion of eicosapentaenoic acid to chain-shortened omega-3 fatty acid metabolites by peroxisomal oxidation [In Process Citation] ; J Lipid Res ; 1998 ; 39(5) ; 978-86
Zhang ; Z ; 6396 ; Regulation of the human long chain acyl-CoA dehydrogenase gene by nuclear hormone receptor transcription factors. ; Biochim Biophys Acta ; 1997 ; 1350(1) ; 53-64