253 related articles for article (PubMed ID: 8356011)
1. 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]
2. Immunochemical characterization of variant long-chain acyl-CoA dehydrogenase in cultured fibroblasts from nine patients with long-chain acyl-CoA dehydrogenase deficiency.
Indo Y; Coates PM; Hale DE; Tanaka K
Pediatr Res; 1991 Sep; 30(3):211-5. PubMed ID: 1945557
[TBL] [Abstract][Full Text] [Related]
3. Gestational, pathologic and biochemical differences between very long-chain acyl-CoA dehydrogenase deficiency and long-chain acyl-CoA dehydrogenase deficiency in the mouse.
Cox KB; Hamm DA; Millington DS; Matern D; Vockley J; Rinaldo P; Pinkert CA; Rhead WJ; Lindsey JR; Wood PA
Hum Mol Genet; 2001 Sep; 10(19):2069-77. PubMed ID: 11590124
[TBL] [Abstract][Full Text] [Related]
4. 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]
5. Molecular basis of very long chain acyl-CoA dehydrogenase deficiency in three Israeli patients: identification of a complex mutant allele with P65L and K247Q mutations, the former being an exonic mutation causing exon 3 skipping.
Watanabe H; Orii KE; Fukao T; Song XQ; Aoyama T; IJlst L; Ruiter J; Wanders RJ; Kondo N
Hum Mutat; 2000; 15(5):430-8. PubMed ID: 10790204
[TBL] [Abstract][Full Text] [Related]
6. Synergistic heterozygosity in mice with inherited enzyme deficiencies of mitochondrial fatty acid beta-oxidation.
Schuler AM; Gower BA; Matern D; Rinaldo P; Vockley J; Wood PA
Mol Genet Metab; 2005 May; 85(1):7-11. PubMed ID: 15862275
[TBL] [Abstract][Full Text] [Related]
7. 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]
8. Disrupted blastocoele formation reveals a critical developmental role for long-chain acyl-CoA dehydrogenase.
Berger PS; Wood PA
Mol Genet Metab; 2004 Aug; 82(4):266-72. PubMed ID: 15308124
[TBL] [Abstract][Full Text] [Related]
9. Genomic DNA organization of human mitochondrial very-long-chain acyl-CoA dehydrogenase and mutation analysis.
Orii KO; Aoyama T; Souri M; Orii KE; Kondo N; Orii T; Hashimoto T
Biochem Biophys Res Commun; 1995 Dec; 217(3):987-92. PubMed ID: 8554625
[TBL] [Abstract][Full Text] [Related]
10. Tissue-specific strategies of the very-long chain acyl-CoA dehydrogenase-deficient (VLCAD-/-) mouse to compensate a defective fatty acid β-oxidation.
Tucci S; Herebian D; Sturm M; Seibt A; Spiekerkoetter U
PLoS One; 2012; 7(9):e45429. PubMed ID: 23024820
[TBL] [Abstract][Full Text] [Related]
11. Acyl-CoA dehydrogenase 9 (ACAD 9) is the long-chain acyl-CoA dehydrogenase in human embryonic and fetal brain.
Oey NA; Ruiter JP; Ijlst L; Attie-Bitach T; Vekemans M; Wanders RJ; Wijburg FA
Biochem Biophys Res Commun; 2006 Jul; 346(1):33-7. PubMed ID: 16750164
[TBL] [Abstract][Full Text] [Related]
12. In vitro characterization and in vivo expression of human very-long chain acyl-CoA dehydrogenase.
Merritt JL; Matern D; Vockley J; Daniels J; Nguyen TV; Schowalter DB
Mol Genet Metab; 2006 Aug; 88(4):351-8. PubMed ID: 16621643
[TBL] [Abstract][Full Text] [Related]
13. A polymorphic variant in the human electron transfer flavoprotein alpha-chain (alpha-T171) displays decreased thermal stability and is overrepresented in very-long-chain acyl-CoA dehydrogenase-deficient patients with mild childhood presentation.
Bross P; Pedersen P; Winter V; Nyholm M; Johansen BN; Olsen RK; Corydon MJ; Andresen BS; Eiberg H; Kolvraa S; Gregersen N
Mol Genet Metab; 1999 Jun; 67(2):138-47. PubMed ID: 10356313
[TBL] [Abstract][Full Text] [Related]
14. A novel disease with deficiency of mitochondrial very-long-chain acyl-CoA dehydrogenase.
Aoyama T; Uchida Y; Kelley RI; Marble M; Hofman K; Tonsgard JH; Rhead WJ; Hashimoto T
Biochem Biophys Res Commun; 1993 Mar; 191(3):1369-72. PubMed ID: 8466512
[TBL] [Abstract][Full Text] [Related]
15. A heterozygous missense mutation in adolescent-onset very long-chain acyl-CoA dehydrogenase deficiency with exercise-induced rhabdomyolysis.
Hisahara S; Matsushita T; Furuyama H; Tajima G; Shigematsu Y; Imai T; Shimohama S
Tohoku J Exp Med; 2015 Apr; 235(4):305-10. PubMed ID: 25843429
[TBL] [Abstract][Full Text] [Related]
16. Immunochemical characterization of variant medium-chain acyl-CoA dehydrogenase in fibroblasts from patients with medium-chain acyl-CoA dehydrogenase deficiency.
Coates PM; Indo Y; Young D; Hale DE; Tanaka K
Pediatr Res; 1992 Jan; 31(1):34-8. PubMed ID: 1594327
[TBL] [Abstract][Full Text] [Related]
17. MS/MS-based newborn and family screening detects asymptomatic patients with very-long-chain acyl-CoA dehydrogenase deficiency.
Spiekerkoetter U; Sun B; Zytkovicz T; Wanders R; Strauss AW; Wendel U
J Pediatr; 2003 Sep; 143(3):335-42. PubMed ID: 14517516
[TBL] [Abstract][Full Text] [Related]
18. Identification of two novel mutations in the hypoglycemic phenotype of very long chain acyl-CoA dehydrogenase deficiency.
He G; Yang BZ; Roe DS; Teramoto R; Aleck K; Grebe TA; Roe CR; Ding JH
Biochem Biophys Res Commun; 1999 Oct; 264(2):483-7. PubMed ID: 10529389
[TBL] [Abstract][Full Text] [Related]
19. Influence of dietary fatty acid chain-length on metabolic tolerance in mouse models of inherited defects in mitochondrial fatty acid beta-oxidation.
Schuler AM; Gower BA; Matern D; Rinaldo P; Wood PA
Mol Genet Metab; 2004 Dec; 83(4):322-9. PubMed ID: 15589119
[TBL] [Abstract][Full Text] [Related]
20. Is autism a disorder of fatty acid metabolism? Possible dysfunction of mitochondrial beta-oxidation by long chain acyl-CoA dehydrogenase.
Clark-Taylor T; Clark-Taylor BE
Med Hypotheses; 2004; 62(6):970-5. PubMed ID: 15142659
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]