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Results: 1 to 20 of 192

Similar articles for PubMed (Select 8466512)

1.

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 31;191(3):1369-72.

PMID:
8466512
2.

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.

3.

Mitochondrial very-long-chain acyl-coenzyme A dehydrogenase deficiency: clinical characteristics and diagnostic considerations in 30 patients.

Vianey-Saban C, Divry P, Brivet M, Nada M, Zabot MT, Mathieu M, Roe C.

Clin Chim Acta. 1998 Jan 12;269(1):43-62.

PMID:
9498103
4.

Prenatal diagnosis of mitochondrial fatty acid oxidation defects.

Nada MA, Vianey-Saban C, Roe CR, Ding JH, Mathieu M, Wappner RS, Bialer MG, McGlynn JA, Mandon G.

Prenat Diagn. 1996 Feb;16(2):117-24.

PMID:
8650121
5.

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.

6.

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.

7.

Purification of human very-long-chain acyl-coenzyme A dehydrogenase and characterization of its deficiency in seven patients.

Aoyama T, Souri M, Ushikubo S, Kamijo T, Yamaguchi S, Kelley RI, Rhead WJ, Uetake K, Tanaka K, Hashimoto T.

J Clin Invest. 1995 Jun;95(6):2465-73.

8.

Very-long-chain acyl-CoA dehydrogenase subunit assembles to the dimer form on mitochondrial inner membrane.

Souri M, Aoyama T, Hoganson G, Hashimoto T.

FEBS Lett. 1998 Apr 17;426(2):187-90.

9.

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. Epub 2005 Feb 16.

PMID:
15862275
10.

Medium-chain and long-chain acyl CoA dehydrogenase deficiency: clinical, pathologic and ultrastructural differentiation from Reye's syndrome.

Treem WR, Witzleben CA, Piccoli DA, Stanley CA, Hale DE, Coates PM, Watkins JB.

Hepatology. 1986 Nov-Dec;6(6):1270-8.

PMID:
3793003
11.

Mutation analysis in mitochondrial fatty acid oxidation defects: Exemplified by acyl-CoA dehydrogenase deficiencies, with special focus on genotype-phenotype relationship.

Gregersen N, Andresen BS, Corydon MJ, Corydon TJ, Olsen RK, Bolund L, Bross P.

Hum Mutat. 2001 Sep;18(3):169-89. Review.

PMID:
11524729
12.

Very long-chain acyl coenzyme A dehydrogenase deficiency presenting with exercise-induced myoglobinuria.

Ogilvie I, Pourfarzam M, Jackson S, Stockdale C, Bartlett K, Turnbull DM.

Neurology. 1994 Mar;44(3 Pt 1):467-73.

PMID:
8145917
13.

Very long chain acyl-CoA dehydrogenase deficiency: identification of a new inborn error of mitochondrial fatty acid oxidation in fibroblasts.

Bertrand C, Largillière C, Zabot MT, Mathieu M, Vianey-Saban C.

Biochim Biophys Acta. 1993 Jan 22;1180(3):327-9.

PMID:
8422439
14.

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.

PMID:
16950999
15.
16.

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.

17.

Long-chain acyl-CoA profiles in cultured fibroblasts from patients with defects in fatty acid oxidation.

Tamvakopoulos CS, Willi S, Anderson VE, Hale DE.

Biochem Mol Med. 1995 Jun;55(1):15-21.

PMID:
7551821
18.
20.

Very long chain acyl-CoA dehydrogenase deficiency in a pair of mildly affected monozygotic twin sister in their late fifties.

Zia A, Kolodny EH, Pastores GM.

J Inherit Metab Dis. 2007 Oct;30(5):817. Epub 2007 May 19.

PMID:
17514507
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