The Identification of a Succinyl-CoA Thioesterase Suggests a Novel Pathway for Succinate Production in Peroxisomes

• Published in: The Journal of biological chemistry Vol. 280; no. 46; pp. 38125 - 38132
• Main Authors: Westin, Maria A.K., Hunt, Mary C., Alexson, Stefan E.H.
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 18.11.2005; American Society for Biochemistry and Molecular Biology
• Subjects: Animals; Blotting, Western; Cloning, Molecular; Dicarboxylic Acids - chemistry; Dicarboxylic Acids - metabolism; Electrophoresis, Polyacrylamide Gel; Endoplasmic Reticulum - metabolism; Fatty Acids - metabolism; Fibroblasts - metabolism; Gene Expression Regulation; Green Fluorescent Proteins - metabolism; Humans; Hydrolysis; Kidney - metabolism; Kinetics; Liver - metabolism; Male; Medicin och hälsovetenskap; Mice; Mice, Transgenic; Models, Biological; Palmitoyl-CoA Hydrolase - chemistry; Palmitoyl-CoA Hydrolase - physiology; Peroxisomes - metabolism; PPAR alpha - metabolism; Pyrimidines - pharmacology; Recombinant Proteins - chemistry; Reverse Transcriptase Polymerase Chain Reaction; RNA, Messenger - metabolism; Substrate Specificity; Succinic Acid - chemistry; Thiolester Hydrolases - chemistry; Thiolester Hydrolases - metabolism; Thiolester Hydrolases - physiology; Time Factors; Tissue Distribution; Up-Regulation

Dicarboxylic acids are formed by ω-oxidation of fatty acids in the endoplasmic reticulum and degraded as the CoA ester via β-oxidation in peroxisomes. Both synthesis and degradation of dicarboxylic acids occur mainly in kidney and liver, and the chain-shortened dicarboxylic acids are excreted in the...