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PEROXISOMAL β-OXIDATION AND PEROXISOME PROLIFERATOR–ACTIVATED RECEPTOR α: An Adaptive Metabolic System
TLDR
The β-Oxidation chain shortening of long-chain and very-long-chain fatty acyl-coenzyme (CoAs) and dicarboxylic acids that serve as substrates for peroxisomal β-oxidation are studied.
Peroxisomal beta-oxidation and peroxisome proliferator-activated receptor alpha: an adaptive metabolic system.
TLDR
Evidence derived from mice deficient in PPAR alpha, peroxisomal fatty acyl-CoA oxidase, and some of the other enzymes of the two per oxisomal beta-oxidation pathways points to the critical importance of PPARalpha and of the classical perox isome proliferator-activated receptor alpha in energy metabolism, and in the development of hepatic steatosis, steatohepatitis, and liver cancer.
Altered Constitutive Expression of Fatty Acid-metabolizing Enzymes in Mice Lacking the Peroxisome Proliferator-activated Receptor α (PPARα)*
TLDR
PPARα modulates constitutive expression of genes encoding several mitochondrial fatty acid-catabolizing enzymes in addition to mediating inducible mitochondrial and peroxisomal fatty acid β-oxidation, thus establishing a role for the receptor in fatty acid homeostasis.
Altered constitutive expression of fatty acid-metabolizing enzymes in mice lacking the peroxisome proliferator-activated receptor alpha (PPARalpha).
TLDR
PPARalpha modulates constitutive expression of genes encoding several mitochondrial fatty acid-catabolizing enzymes in addition to mediating inducible mitochondrial and peroxisomal fatty acid beta-oxidation, thus establishing a role for the receptor in fatty acid homeostasis.
Defect in Peroxisome Proliferator-activated Receptor α-inducible Fatty Acid Oxidation Determines the Severity of Hepatic Steatosis in Response to Fasting*
TLDR
Observations point to the critical importance of PPARα in the transcriptional regulatory responses to fasting and in determining the severity of hepatic steatosis.
Insertion of the 70-kDa Peroxisomal Membrane Protein into Peroxisomal Membranes in Vivo and in Vitro(*)
TLDR
PMP70 is post-translationally transported to peroxisomes without processing and inserted into peroxISomal membranes by a specific mechanism in which a proteinaceous receptor and a certain internal sequence of PMP70 are involved.
Molecular cloning and sequence of the complementary DNA encoding human mitochondrial acetoacetyl-coenzyme A thiolase and study of the variant enzymes in cultured fibroblasts from patients with
TLDR
The results suggest that different mechanisms are involved in the enzyme defects in the four patients with 3-ketothiolase deficiency, and that the T2 proteins in the fibroblasts from these patients are present in the mitochondria.
Structure and regulation of rat long-chain acyl-CoA synthetase.
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