292 related articles for article (PubMed ID: 20816094)
21. Relationship between structure and substrate-chain-length specificity of mitochondrial very-long-chain acyl-coenzyme A dehydrogenase.
Souri M; Aoyama T; Yamaguchi S; Hashimoto T
Eur J Biochem; 1998 Nov; 257(3):592-8. PubMed ID: 9839948
[TBL] [Abstract][Full Text] [Related]
22. Neonatal multiorgan failure due to ACAD9 mutation and complex I deficiency with mitochondrial hyperplasia in liver, cardiac myocytes, skeletal muscle, and renal tubules.
Leslie N; Wang X; Peng Y; Valencia CA; Khuchua Z; Hata J; Witte D; Huang T; Bove KE
Hum Pathol; 2016 Mar; 49():27-32. PubMed ID: 26826406
[TBL] [Abstract][Full Text] [Related]
23. Structural basis for expanded substrate specificities of human long chain acyl-CoA dehydrogenase and related acyl-CoA dehydrogenases.
Narayanan B; Xia C; McAndrew R; Shen AL; Kim JP
Sci Rep; 2024 Jun; 14(1):12976. PubMed ID: 38839792
[TBL] [Abstract][Full Text] [Related]
24. Molecular heterogeneity in very-long-chain acyl-CoA dehydrogenase deficiency causing pediatric cardiomyopathy and sudden death.
Mathur A; Sims HF; Gopalakrishnan D; Gibson B; Rinaldo P; Vockley J; Hug G; Strauss AW
Circulation; 1999 Mar; 99(10):1337-43. PubMed ID: 10077518
[TBL] [Abstract][Full Text] [Related]
25. [Study of the inborn errors of mitochondrial fatty acid beta-oxidation deficiency].
Zhu JM; Yang Z
Beijing Da Xue Xue Bao Yi Xue Ban; 2006 Apr; 38(2):214-7. PubMed ID: 16617370
[TBL] [Abstract][Full Text] [Related]
26. Identification of the catalytic base in long chain acyl-CoA dehydrogenase.
Djordjevic S; Dong Y; Paschke R; Frerman FE; Strauss AW; Kim JJ
Biochemistry; 1994 Apr; 33(14):4258-64. PubMed ID: 8155643
[TBL] [Abstract][Full Text] [Related]
27. Molecular evolution and substrate specificity of acyl-CoA dehydrogenases: chimaeric "medium/long' chain-specific enzyme from medium-chain acyl-CoA dehydrogenase.
Nandy A; Küchler B; Ghisla S
Biochem Soc Trans; 1996 Feb; 24(1):105-10. PubMed ID: 8674584
[No Abstract] [Full Text] [Related]
28. Regulation of fatty acid transport protein and mitochondrial and peroxisomal beta-oxidation gene expression by fatty acids in developing rats.
Ouali F; Djouadi F; Merlet-Bénichou C; Riveau B; Bastin J
Pediatr Res; 2000 Nov; 48(5):691-6. PubMed ID: 11044493
[TBL] [Abstract][Full Text] [Related]
29. Evidence of a wide spectrum of cardiac involvement due to ACAD9 mutations: Report on nine patients.
Dewulf JP; Barrea C; Vincent MF; De Laet C; Van Coster R; Seneca S; Marie S; Nassogne MC
Mol Genet Metab; 2016 Jul; 118(3):185-189. PubMed ID: 27233227
[TBL] [Abstract][Full Text] [Related]
30. Understanding mitochondrial complex I assembly in health and disease.
Mimaki M; Wang X; McKenzie M; Thorburn DR; Ryan MT
Biochim Biophys Acta; 2012 Jun; 1817(6):851-62. PubMed ID: 21924235
[TBL] [Abstract][Full Text] [Related]
31. Long-chain acyl-CoA dehydrogenase is a key enzyme in the mitochondrial beta-oxidation of unsaturated fatty acids.
Lea W; Abbas AS; Sprecher H; Vockley J; Schulz H
Biochim Biophys Acta; 2000 May; 1485(2-3):121-8. PubMed ID: 10832093
[TBL] [Abstract][Full Text] [Related]
32. Exome sequencing identifies ACAD9 mutations as a cause of complex I deficiency.
Haack TB; Danhauser K; Haberberger B; Hoser J; Strecker V; Boehm D; Uziel G; Lamantea E; Invernizzi F; Poulton J; Rolinski B; Iuso A; Biskup S; Schmidt T; Mewes HW; Wittig I; Meitinger T; Zeviani M; Prokisch H
Nat Genet; 2010 Dec; 42(12):1131-4. PubMed ID: 21057504
[TBL] [Abstract][Full Text] [Related]
33. De novo fatty acid biosynthesis and elongation in very long-chain acyl-CoA dehydrogenase-deficient mice supplemented with odd or even medium-chain fatty acids.
Tucci S; Behringer S; Spiekerkoetter U
FEBS J; 2015 Nov; 282(21):4242-53. PubMed ID: 26284828
[TBL] [Abstract][Full Text] [Related]
34. Bezafibrate increases very-long-chain acyl-CoA dehydrogenase protein and mRNA expression in deficient fibroblasts and is a potential therapy for fatty acid oxidation disorders.
Djouadi F; Aubey F; Schlemmer D; Ruiter JP; Wanders RJ; Strauss AW; Bastin J
Hum Mol Genet; 2005 Sep; 14(18):2695-703. PubMed ID: 16115821
[TBL] [Abstract][Full Text] [Related]
35. Structure and mechanism of action of the acyl-CoA dehydrogenases.
Thorpe C; Kim JJ
FASEB J; 1995 Jun; 9(9):718-25. PubMed ID: 7601336
[TBL] [Abstract][Full Text] [Related]
36. Neonatal screening for very long-chain acyl-coA dehydrogenase deficiency: enzymatic and molecular evaluation of neonates with elevated C14:1-carnitine levels.
Liebig M; Schymik I; Mueller M; Wendel U; Mayatepek E; Ruiter J; Strauss AW; Wanders RJ; Spiekerkoetter U
Pediatrics; 2006 Sep; 118(3):1065-9. PubMed ID: 16950999
[TBL] [Abstract][Full Text] [Related]
37. Identification of very-long-chain acyl-CoA dehydrogenase deficiency in three patients previously diagnosed with long-chain acyl-CoA dehydrogenase deficiency.
Yamaguchi S; Indo Y; Coates PM; Hashimoto T; Tanaka K
Pediatr Res; 1993 Jul; 34(1):111-3. PubMed ID: 8356011
[TBL] [Abstract][Full Text] [Related]
38. Medium-chain acyl-CoA dehydrogenase deficiency in children with non-ketotic hypoglycemia and low carnitine levels.
Stanley CA; Hale DE; Coates PM; Hall CL; Corkey BE; Yang W; Kelley RI; Gonzales EL; Williamson JR; Baker L
Pediatr Res; 1983 Nov; 17(11):877-84. PubMed ID: 6646897
[TBL] [Abstract][Full Text] [Related]
39. Oxidation of unsaturated fatty acids by human fibroblasts with very-long-chain acyl-CoA dehydrogenase deficiency: aspects of substrate specificity and correlation with clinical phenotype.
Roe DS; Vianey-Saban C; Sharma S; Zabot MT; Roe CR
Clin Chim Acta; 2001 Oct; 312(1-2):55-67. PubMed ID: 11580910
[TBL] [Abstract][Full Text] [Related]
40. Assembly defects of multiple respiratory chain complexes in a child with cardiac hypertrophy associated with a novel ACAD9 mutation.
Fragaki K; Chaussenot A; Boutron A; Bannwarth S; Rouzier C; Chabrol B; Paquis-Flucklinger V
Mol Genet Metab; 2017 Jul; 121(3):224-226. PubMed ID: 28529009
[TBL] [Abstract][Full Text] [Related]
[Previous] [Next] [New Search]
