Article

Molecular and Cellular Biochemistry

, Volume 167, Issue 1, pp 1-29

First online:

Biochemistry of peroxisomes in health and disease

  • Inderjit SinghAffiliated withDepartment of Pediatrics, Anatomy and Cell Biology, Medical University of South Carolina

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Abstract

The ubiquitous distribution of peroxisomes and the identification of a number of inherited diseases associated with peroxisomal dysfunction indicate that peroxisomes play an essential part in cellular metabolism. Some of the most important metabolic functions of peroxisomes include the synthesis of plasmalogens, bile acids, cholesterol and dolichol, and the oxidation of fatty acids (very long chain fatty acids > C22, branched chain fatty acids (e.g. phytanic acid), dicarboxylic acids, unsaturated fatty acids, prostaglandins, pipecolic acid and glutaric acid). Peroxisomes are also responsible for the metabolism of purines, polyamines, amino acids, glyoxylate and reactive oxygen species (e.g. O-2 and H2O2). Peroxisomal diseases result from the dysfunction of one or more peroxisomal metabolic functions, the majority of which manifest as neurological abnormalities. The quantitation of peroxisomal metabolic functions (e.g. levels of specific metabolites and/or enzyme activity) has bec ome the basis of clinical diagnosis of diseases associated with the organelle. The study of peroxisomal diseases has also contributed towards the further elucidation of a number of metabolic functions of peroxisomes. (Mol Cell Biochem 167:1-29, 1997)

peroxisomes peroxisomal diseases fatty acid β-oxidation fatty acid α-oxidation bile acids and plasmalogen H2O2 metabolism