A unique, membrane-bound, multifunctional enzyme from human liver mitochondria catalysing three steps of fatty acid beta-oxidation.

KAREN CARPENTER, RODNEY h4IDDElDN multifunctional enzyme Pure LCHAD. with specific activity 30.4pmol 3-ketohexacatalysing three steps of decanoyl-CoA/min/mg, also showed ECH and KAT activities of 92.9pmol 2-octenoyl-CoA/min/mg and 4 . 3 ~ 0 1 J-ketodecanoylCoA/min/mg respectively. All 3 enzymes were active with acyl-CoA J. POLLI’lT* and BRUCE substrates of chain length from Q to c16 but no significant activity was detectable with substrates. Therefore “LCHAD” appears to be a multifunctional B-oxidation enzyme of lonn-chain specificity and Department of Biochemistry. Nottingham University Medical School, Nottingham NG7 2UH, U.K. and *Department of Paediatrics, Childrens Hospital, Sheffield S10 2TH, U.K.