[Study of the inborn errors of mitochondrial fatty acid beta-oxidation deficiency].

Abstract

Mitochondrial fatty acids beta-oxidation is a repetitive process of four steps which provides the major source of energy for heart, liver and skeletal muscle. Several enzymes are involved in this spiral cycle. The medium-chain acyl-CoA dehydrogenase (MCAD), the short-chain acyl-CoA dehydrogenase (SCAD), the long-chain 3-hydroxy acyl-CoA dehydrogenase (LCHAD) and the carnitine-palmitoyl-CoA transferase II (CPT II) deficiency have been recognized as the most common inborn errors of metabolism and frequently reported in their association with sudden infant death (SID). The prevalent mutations in these genes need further investigation in different populations.

• Authors: Jin-ming Zhu, Zi Yang
• Journal: Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences (Beijing Da Xue Xue Bao Yi Xue Ban) Vol. 38 Issue 2 Pg. 214-7 (Apr 18 2006) ISSN: 1671-167X [Print] China
• PMID: 16617370 (Publication Type: English Abstract, Journal Article, Research Support, Non-U.S. Gov't, Review)

Chemical References

• Fatty Acids
• Acyl-CoA Dehydrogenases

Topics

• Acyl-CoA Dehydrogenases (metabolism)
• Fatty Acids (metabolism)
• Humans
• Lipid Metabolism, Inborn Errors (genetics, metabolism)
• Mitochondria (metabolism)
• Oxidation-Reduction