Long chain fatty acids penetrate the inner mitochondrial membrane as?
Correct Answer: Carnitine derivatives
Description: Long-Chain Fatty Acids Penetrate the Inner Mitochondrial Membrane as Carnitine Derivatives
Carnitine ( -hydroxy- -trimethylammonium butyrate), (CH3)3N+—CH2—CH(OH)—CH2—COO–, is widely distributed and is particularly abundant in muscle. Long-chain acyl-CoA (or FFA) cannot penetrate the inner membrane of mitochondria. In the presence of carnitine, however, carnitine palmitoyltransferase-I, located in the outer mitochondrial membrane, converts long-chain acyl-CoA to acylcarnitine, which is able to penetrate the inner membrane and gain access to the -oxidation system of enzymes. Carnitine-acylcarnitine translocase acts as an inner membrane exchange transporter. Acylcarnitine is transported in, coupled with the transport out of one molecule of carnitine. The acylcarnitine then reacts with CoA, catalyzed by carnitine palmitoyltransferase-II, located on the inside of the inner membrane, reforming acyl-CoA in the mitochondrial matrix, and carnitine is liberated.
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