Metabolism
Research from University of Pittsburgh, Department of Pediatrics broadens understanding of metabolism
Published in Genomics and Genetics Weekly, July 13th, 2007
Current study results from the report, "Expression and characterization of mutations in human very long-chain acyl-CoA dehydrogenase using a prokaryotic system," have been published. "Very long-chain acyl-CoA dehydrogenase (VLCAD) catalyzes the first enzymatic step in the mitochondrial beta-oxidation of fatty acids 14-20 carbons in length. More than 100 cases of VLCAD deficiency have been reported with the disease varying from a severe, often fatal neonatal form to a mild adult-onset form," investigators in the United States report.
"VLCAD is distinguished from matrix-soluble acyl-CoA dehydrogenases by its unique C-terminal domain, homodimeric structure, and...
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