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Results: 1 to 20 of 101

1.

Expression and characterization of mutations in human very long-chain acyl-CoA dehydrogenase using a prokaryotic system.

Goetzman ES, Wang Y, He M, Mohsen AW, Ninness BK, Vockley J.

Mol Genet Metab. 2007 Jun;91(2):138-47. Epub 2007 Mar 19.

PMID:
17374501
[PubMed - indexed for MEDLINE]
Free PMC Article
3.

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 1;257(3):592-8.

PMID:
9839948
[PubMed - indexed for MEDLINE]
Free Article
4.

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. Epub 2012 Sep 14.

PMID:
23024820
[PubMed - indexed for MEDLINE]
Free PMC Article
5.

Catalytic and FAD-binding residues of mitochondrial very long chain acyl-coenzyme A dehydrogenase.

Souri M, Aoyama T, Cox GF, Hashimoto T.

J Biol Chem. 1998 Feb 13;273(7):4227-31.

PMID:
9461620
[PubMed - indexed for MEDLINE]
Free Article
6.

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 15;10(19):2069-77.

PMID:
11590124
[PubMed - indexed for MEDLINE]
Free Article
7.

Structural basis for substrate fatty acyl chain specificity: crystal structure of human very-long-chain acyl-CoA dehydrogenase.

McAndrew RP, Wang Y, Mohsen AW, He M, Vockley J, Kim JJ.

J Biol Chem. 2008 Apr 4;283(14):9435-43. doi: 10.1074/jbc.M709135200. Epub 2008 Jan 28.

PMID:
18227065
[PubMed - indexed for MEDLINE]
Free PMC Article
8.

Diagnosis of very long chain acyl-dehydrogenase deficiency from an infant's newborn screening card.

Wood JC, Magera MJ, Rinaldo P, Seashore MR, Strauss AW, Friedman A.

Pediatrics. 2001 Jul;108(1):E19.

PMID:
11433098
[PubMed - indexed for MEDLINE]
9.

Molecular and cellular pathology of very-long-chain acyl-CoA dehydrogenase deficiency.

Schiff M, Mohsen AW, Karunanidhi A, McCracken E, Yeasted R, Vockley J.

Mol Genet Metab. 2013 May;109(1):21-7. doi: 10.1016/j.ymgme.2013.02.002. Epub 2013 Feb 13.

PMID:
23480858
[PubMed - indexed for MEDLINE]
Free PMC Article
10.

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.

Andresen BS, Bross P, Vianey-Saban C, Divry P, Zabot MT, Roe CR, Nada MA, Byskov A, Kruse TA, Neve S, Kristiansen K, Knudsen I, Corydon MJ, Gregersen N.

Hum Mol Genet. 1996 Apr;5(4):461-72. Erratum in: Hum Mol Genet 1996 Sep;5(9):1390.

PMID:
8845838
[PubMed - indexed for MEDLINE]
Free Article
11.

Molecular basis of human mitochondrial very-long-chain acyl-CoA dehydrogenase deficiency causing cardiomyopathy and sudden death in childhood.

Strauss AW, Powell CK, Hale DE, Anderson MM, Ahuja A, Brackett JC, Sims HF.

Proc Natl Acad Sci U S A. 1995 Nov 7;92(23):10496-500.

PMID:
7479827
[PubMed - indexed for MEDLINE]
Free PMC Article
12.

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 15;14(18):2695-703. Epub 2005 Aug 22.

PMID:
16115821
[PubMed - indexed for MEDLINE]
Free Article
13.

Genetic basis for correction of very-long-chain acyl-coenzyme A dehydrogenase deficiency by bezafibrate in patient fibroblasts: toward a genotype-based therapy.

Gobin-Limballe S, Djouadi F, Aubey F, Olpin S, Andresen BS, Yamaguchi S, Mandel H, Fukao T, Ruiter JP, Wanders RJ, McAndrew R, Kim JJ, Bastin J.

Am J Hum Genet. 2007 Dec;81(6):1133-43. Epub 2007 Oct 29.

PMID:
17999356
[PubMed - indexed for MEDLINE]
Free PMC Article
14.

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.

PMID:
10356313
[PubMed - indexed for MEDLINE]
15.

In vitro characterization and in vivo expression of human very-long chain acyl-CoA dehydrogenase.

Merritt JL 2nd, Matern D, Vockley J, Daniels J, Nguyen TV, Schowalter DB.

Mol Genet Metab. 2006 Aug;88(4):351-8. Epub 2006 Apr 18.

PMID:
16621643
[PubMed - indexed for MEDLINE]
16.

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.

PMID:
8356011
[PubMed - indexed for MEDLINE]
17.

Compared effects of missense mutations in Very-Long-Chain Acyl-CoA Dehydrogenase deficiency: Combined analysis by structural, functional and pharmacological approaches.

Gobin-Limballe S, McAndrew RP, Djouadi F, Kim JJ, Bastin J.

Biochim Biophys Acta. 2010 May;1802(5):478-84. doi: 10.1016/j.bbadis.2010.01.001. Epub 2010 Jan 12.

PMID:
20060901
[PubMed - indexed for MEDLINE]
Free PMC Article
18.

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.

PMID:
14517516
[PubMed - indexed for MEDLINE]
19.

Long-term correction of very long-chain acyl-coA dehydrogenase deficiency in mice using AAV9 gene therapy.

Keeler AM, Conlon T, Walter G, Zeng H, Shaffer SA, Dungtao F, Erger K, Cossette T, Tang Q, Mueller C, Flotte TR.

Mol Ther. 2012 Jun;20(6):1131-8. doi: 10.1038/mt.2012.39. Epub 2012 Mar 6.

PMID:
22395529
[PubMed - indexed for MEDLINE]
Free PMC Article
20.

Human acyl-CoA dehydrogenase-9 plays a novel role in the mitochondrial beta-oxidation of unsaturated fatty acids.

Ensenauer R, He M, Willard JM, Goetzman ES, Corydon TJ, Vandahl BB, Mohsen AW, Isaya G, Vockley J.

J Biol Chem. 2005 Sep 16;280(37):32309-16. Epub 2005 Jul 14.

PMID:
16020546
[PubMed - indexed for MEDLINE]
Free Article

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