Journal
AMERICAN JOURNAL OF PHYSIOLOGY-GASTROINTESTINAL AND LIVER PHYSIOLOGY
Volume 281, Issue 6, Pages G1333-G1339Publisher
AMER PHYSIOLOGICAL SOC
DOI: 10.1152/ajpgi.2001.281.6.G1333
Keywords
peroxisomes; fatty acid beta- and omega-oxidation; peroxisomal proliferator-activated receptor alpha; peroxisomal biogenesis disorders
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Peroxisomes are involved in the beta -oxidation chain shortening of long-chain and very-long-chain fatty acyl-CoAs, long-chain dicarboxylyl-CoAs, the CoA esters of eicosanoids, 2-methyl-branched fatty acyl-CoAs, and the CoA esters of the bile acid intermediates, and in the process, they generate H2O2. There are two complete sets of beta -oxidation enzymes present in peroxisomes, with each set consisting of three distinct enzymes. The classic PPAR alpha -regulated and inducible set participates in the beta -oxidation of straight-chain fatty acids, whereas the second noninducible set acts on branched-chain fatty acids. Long-chain and very-long-chain fatty acids are also metabolized by the cytochrome P-450 CYP4A omega -oxidation system to dicarboxylic acids that serve as substrates for peroxisomal beta -oxidation. Evidence derived from mouse models of PPAR alpha and peroxisomal beta -oxidation deficiency highlights the critical importance of the defects in PPAR alpha- inducible beta -oxidation in energy metabolism and in the development of steatohepatitis.
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