Effects of cardiolipin on carnitine palmitoyltransferase kinetics
نویسندگان
چکیده
منابع مشابه
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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I t is generally accepted that the mitochondria1 carnitine palmitoyltransferases play an important role in the regulation of hepatic fatty acid oxidation [ I ] . These two enzymes express their activities on either side of the mitochondrial inner membrane that acts as a barrier to the transfer of fatty acids into the mitochondrial matrix. The enzyme that lies outsidc this barrier is located on ...
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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...
متن کاملInteracting effects of L-carnitine and malonyl-CoA on rat liver carnitine palmitoyltransferase.
Malonyl-CoA significantly increased the Km for L-carnitine of overt carnitine palmitoyltransferase in liver mitochondria from fed rats. This effect was observed when the molar palmitoyl-CoA/albumin concentration ratio was low (0.125-1.0), but not when it was higher (2.0). In the absence of malonyl-CoA, the Km for L-carnitine increased with increasing palmitoyl-CoA/albumin ratios. Malonyl-CoA di...
متن کاملFunctional and Structural Basis of Carnitine Palmitoyltransferase
Carnitine palmitoyltransferase 1A (CPT1A) is the key regulatory enzyme of hepatic long-chain fatty acid -oxidation. Human CPT1A deficiency is characterized by recurrent attacks of hypoketotic hypoglycemia. We presently analyzed at both the functional and structural levels five missense mutations identified in three CPT1A-deficient patients, namely A275T, A414V, Y498C, G709E, and G710E. Heterolo...
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ژورنال
عنوان ژورنال: The FASEB Journal
سال: 2006
ISSN: 0892-6638,1530-6860
DOI: 10.1096/fasebj.20.4.a88-d