Premium
The long‐chain 3‐hydroxyacyl‐CoA dehydrogenase of human liver mitochondria
Author(s) -
Carpenter K.,
Middleton B.,
Pollitt R. J.
Publication year - 1991
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1007/bf01811693
Subject(s) - boulevard , queen (butterfly) , library science , postage stamps , biochemistry , medical school , medicine , art , medical education , engineering , biology , art history , pathology , computer science , hymenoptera , botany , civil engineering
The mitochondrial/?-oxidation of fatty acids in neonatal liver is of major importance in energy generation, especially during milk feeding. Inherited disorders of mitochondrial/~-oxidation are life-threatening and relatively common (Pollitt, 1989), but are difficult to diagnose. In part this is because they could be caused by the absence of any of the chain-length-specific enzymes catalysing the four steps of the pathway. Our aim was to establish that human liver mitochondria contain long- and shortchain-specific 3-hydroxyacyl-CoA dehydrogenases. The short-chain-specific enzyme has been extensively studied but little is known about the long-chain enzyme. Absence of the latter from liver is suggested as a cause of some cases of hypoglycaemic 3hydroxydicarboxylic aciduria (Pollitt, 1990).