The kinetics of activation of saturated fatty acids by long chain acyl-CoA synthetase from rat liver microsomes has been studied with a method of selective extraction of free fatty acids based on the insolubility of acyl-CoA in diethyl ether. Saturated fatty acids with a chain length ranging from Cl* to Co were assayed at concentrations varying from 0.5 to 10 PM. Under these conditions, Vm,, is...

The YBR159w gene encodes the major 3-ketoreductase activity of the elongase system of enzymes required for very long-chain fatty acid (VLCFA) synthesis. Mutants lacking the YBR159w gene display many of the phenotypes that have previously been described for mutants with defects in fatty acid elongation. These phenotypes include reduced VLCFA synthesis, accumulation of high levels of dihydrosphin...

Human cytomegalovirus hijacks host cell metabolism, increasing the flux of carbon from glucose to malonyl-CoA, the committed precursor to fatty acid synthesis and elongation. Inhibition of acetyl-CoA carboxylase blocks the production of progeny virus. To probe further the role of fatty acid metabolism during infection, we performed an siRNA screen to identify host cell metabolic enzymes needed ...

zellweger syndrome (zs) is a peroxisomal disorder with a multiple congenital anomalies, characterized by stereotypical facies, profound hypotonia, organ involvement including cerebral, retinal, hepatic, and renal. herein, a 3-month-old female with zs is presented who was referred because of increased liver enzymes (subclinical hepatitis), which was detected in work-up of her neck cyst, severe h...

You, J., X. Zhao, Y. Suo, Y. Li, H. Wang, and G. Chen, Determination of LongChain Fatty Acids in Bryophyte Plant Extracts by HPLC with Fluorescence Detection and Identification with MS, J. Chromatogr. B 848:283–291, 2007. A sensitive method has been developed for the determination of long-chain fatty acids (LCFAs) (>C20) using 1-[2-(ptoluenesulfonate)-ethyl]-2-phenylimidazole-[4,5-f]-9,10-phena...

Fatty acids with carbon chain lengths greater than 22 (VLCFA) have been detected in boar, ram, bull and human spermatozoa. Saturated and mono-unsaturated fatty acids were present in all spermatozoa but, except for human spermatozoa, polyenoic fatty acids were quantitatively the most important components. Marked differences in polyenoic fatty acid composition were observed. Whereas human spermat...

Human skin fibroblasts in suspension are able to degrade [1-'4C]-labeled aand y-methyl branched chain fatty acids such as pristanic and homophytanic acid. Pristanic acid was converted to propionyl-CoA, whereas homophytanic acid was @-oxidized to acetyl-coA. Incubation of skin fibroblasts with [l-i4C]-labeled fatty acids for longer periods produced radiolabeled carbon dioxide, presumably by furt...

zellweger syndrome (zs) is a peroxisomal disorder with a multiple congenital anomalies, characterized by stereotypical facies, profound hypotonia, organ involvement including cerebral, retinal, hepatic, and renal. herein, a 3-month-old female with zs is presented who was referred because of increased liver enzymes (subclinical hepatitis), which was detected in work-up of her neck cyst, severe h...

In mammals, the fatty acid transport proteins (FATP1 through FATP6) are members of a highly conserved family of proteins, which function in fatty acid transport proceeding through vectorial acylation and in the activation of very long chain fatty acids, branched chain fatty acids and secondary bile acids. FATP1, 2 and 4, for example directly function in fatty acid transport and very long chain ...