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Activation - reaction catalyzed by carnitine...

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Activation Fatty acids are activated by fatty acyl CoA synthetase. The reaction: R-COOH + CoASH + ATP <--> R-CO-SCoA + AMP + PP i The subsequent hydrolysis of PPi draws the reaction in the forward direction, maintaining a low cytosolic free fatty acid concentration: PP i + H 2 O --> 2 P i The reaction occurs in the endoplasmic reticulum and the outer mitochondrial membrane. Transport The fatty acyl group is transported into the mitochondrial matrix, where it undergoes beta-oxidation. In the intermembrane space of the mitochondria, fatty acyl CoA reacts with carnitine in a
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Unformatted text preview: reaction catalyzed by carnitine acyltransferase I (CAT-I), yielding CoA and fatty acyl carnitine. The resulting fatty acyl carnitine crosses the inner mitochondrial membrane. • CAT-I is associated with the inner leaflet of the outer mitochondrial membrane. • In liver the CAT-I reaction is rate-limiting; the enzyme is allosterically inhibited by malonyl CoA. Malonyl CoA concentration would be high during fatty acid synthesis. Inhibition of CAT-I by malonyl CoA prevents simultaneous synthesis and degradation of fatty acids....
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