Active sites residues of beef liver carnitine octanoyltransferase (COT) and carnitine palmitoyltransferase (CPT-II)

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Structural Model of the Catalytic Core of Carnitine Palmitoyltransferase I and Carnitine Octanoyltransferase (COT)

Carnitine palmitoyltransferase I (CPT I) and carnitine octanoyltransferase (COT) catalyze the conversion of longand medium-chain acyl-CoA to acylcarnitines in the presence of carnitine. We propose a common threedimensional structural model for the catalytic domain of both, based on fold identification for 200 amino acids surrounding the active site through a threading approach. The model is bas...

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Crystal structure of rat carnitine palmitoyltransferase II (CPT-II).

Carnitine palmitoyltransferase II (CPT-II) has a crucial role in the beta-oxidation of long-chain fatty acids in mitochondria. We report here the crystal structure of rat CPT-II at 1.9A resolution. The overall structure shares strong similarity to those of short- and medium-chain carnitine acyltransferases, although detailed structural differences in the active site region have a significant im...

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Carnitine palmitoyltransferase II deficiency

The authors investigated 32 patients with the muscle form of CPT II deficiency. Total carnitine palmitoyltransferase enzyme system (CPT) activity was normal but abnormally inhibited by malonyl-CoA, palmitoyl-CoA, and the detergents Triton X and Tween 20. Mutation analysis identified three described mutations (S113L, P50H, and F448L) and two novel mutations (M214T and Y479F). Using modeling tech...

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Effects of fasting and malonyl CoA on the kinetics of carnitine palmitoyltransferase and carnitine octanoyltransferase in intact rat liver mitochondria.

There has been considerable interest in the observation that the overt form of carnitine palmitoyltransferase (CPT1) in liver mitochondria is potently inhibited by malonyl CoA [I ,2]. It has been suggested [2] that this is a competitive type of inhibition against long chain acyl CoA substrates. However, this is based upon measurements of ketogenesis [3-51 or indirect calculations of CPT, activi...

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Over-expression and characterization of active recombinant rat liver carnitine palmitoyltransferase II using baculovirus.

The cDNA encoding rat liver carnitine palmitoyltransferase II (CPT-II) was heterologously expressed using a recombinant baculovirus/insect cell system. Unlike Escherichia coli, the baculovirus-infected insect cells expressed mostly soluble active recombinant CPT-II (rCPT-II). CPT activity from crude lysates of recombinant baculovirus-infected insect cells was maximal between 50 and 72 h post-in...

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ژورنال

عنوان ژورنال: Biochemical Journal

سال: 1998

ISSN: 0264-6021,1470-8728

DOI: 10.1042/bj3301029