Acyl-CoA very long-chain

Fatty acid transport protein paralogues 1-6 FATP 1-6 Gene symbols SLC27A1-6 Solute carrier family 27A Very long-chain acyl-CoA synthetase VLCS... 

Venus Flytrap Module Very Long-chain Acyl-CoA Synthetase Very Low-density Lipoprotein Vesicle... 

Enzymes 7,9, and 13 form a trifunctional protein associated with the inner face of the inner mitochondrial membrane. Very-long-chain acyl-CoA dehydrogenase is also associated with other inner mitochondrial membranes while the other enzymes are in the matrix and may be loosely associated with the inner face of the inner membrane. A medium-chain 2-enoyl-CoA hydratase may also be present in the mitochondrial matrix. 

Herrmann, T., et al. Mouse fatty acid transport protein 4 (FATP4) characterization of the gene and functional assessment as a very long chain acyl-CoA synthetase. Gene 2001, 270,... 

Very-long-chain acyl-CoA dehydrogenase and trifunctional protein are the two inner membrane-bound... 

A separate very long-chain-acyl-CoA synthetase is present in peroxisomes for the activation of very long-chain fatty acids, such as arachidonate (20 carbon atoms). These fatty acids are degraded exclusively in the peroxisomes. 

Roe DS, Vianey-Saban C, SharmaS, ZabotMT, Roe CR (2001) 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. Clin Chim Acta 312 55-67... 

Heinzer AK, Watkins PA, Lu IF, Kemp S, Moser AB, Li YY, MihaUk S, Powers JM, Smith KD. A very long-chain acyl-CoA synthetase-deficient mouse and its relevance to X-Unked adrenoleukodystrophy. Hum. Mol. Genet. 2003 12 1145-1154. 

Very Long-Chain Acyl-CoA Dehydrogenase Deficiency... 

Very-long-chain acyl-CoA dehydrogenase (VLCAD) deficiency is an autosomal recessive condition. In the mid 1980s, patients with VLCAD deficiency were initially described as having long-chain acyl-CoA dehydrogenase (LCAD) deficiency. However, without exception, once the VLCAD protein was identified, affected patients were shown to have no detectable VLCAD protein by immunobiot analy-sis or to harbor disease-causing mutations in the VLCAD... 

Figure 55-14 Plasma profiles of plasma acylcarnitine butyl-ester derivatives. A, Normal control. B, Propionic acidemia. C, Short-chain acyl-CoA dehydrogenase deficiency. D, Isovaleric acidemia. E, Medium-chain acyl-CoA dehydrogenase deficiency. F, Very long-chain acyl-CoA dehydrogenase deficiency. G, Long-chain L-3-hydroxy acyl-CoA dehydrogenase deficiency.The symbol marks internal standards [ Hjj-acetylcarnitine (m/z 263) [ HaJ-propionylcarnitine (m/z 277) fH ]-butyrylcarnitlne (m/z 295) pHal-octanoylcarnitine (m/z 347) [ Haj-dodecanoylcarnltine (m/z 403) [ Haj-palmitoy I carnitine (m/z 459).

VLCAD deficiency 201475 Very long-chain acyl-CoA dehydrogenase >1 75,000 Spectrurn from early onset cardiomyopathy, coma, liver disease to adult onset myopathy ... 

Andresen BS, Olpin S, Poorthuis BJ, Scholte HR, Vianey-Saban C, Wanders R, et al. Clear correlation of genotype with disease phenotype in very-long-chain acyl-CoA dehydrogenase deficiency. Am J Hum Genet 1999 64 479-94. 

Aoyama T, Ueno I, Kamijo T, Hashimoto T. Rat very-long-chain acyl-CoA dehydrogenase, a novel mitochondrial acyl-CoA dehydrogenase gene product, is a rate-limiting enzyme in long-chain fatty acid beta-oxidation system. cDNA and deduced amino acid sequence and distinct specificities of the cDNA-expressed protein. J Biol Chem 1994 269 19088-94. 

Uchida Y, Kondo N, Orii T, Hashimoto T. Purification and properties of rat liver peroxisomal very-long-chain acyl-CoA synthetase. J Biochem 1996 119 565-71. 

Spiekerkoetter U, Sun B, Zytkovicz T, Wanders R, Strauss AW, Wendel U. MS/MS-hased newhom and family screening deteds asymptomatic patients with very-long-chain acyl-CoA dehydrogenase defidency. J Pediatr. 2003 Sep 143(3) 335-42. PubMed dtation... 

Touma EH, Rashed MS, Vianey-Saban C, Sakr A, Divry P, Gregersen N, Andresen BS. A severe genotype with favourable outcome in very long chain acyl-CoA dehydrogenase defidency. Arch Dis Child. 2001 Jan 84(l) 58-60. PubMed dtation... 

Souri M, Aoyama T, Hoganson G, Hashimoto T. Very-long-chain acyl-CoA dehydrogenase subunit assembles to the dimer form on mitochondrial inner membrane. FEBS Lett. 1998 Apr 17 426(2) 187-90. PubMed dtation... 

This first step is catalyzed by three isozymes of acyl-CoA dehydrogenase, each specific for a range of fatty-acyl chain lengths very-long-chain acyl-CoA dehydrogenase (VLCAD), acting on fatty acids of 12 to 18... 

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