peroxisome

The peroxisome is a cellular organelle bounded by a single-membrane that is present in most eukaryotic cells. Beta oxidation of long and very long chain fatty acids occurs in the peroxisome. The organelle is also involved in bile acid synthesis, cholesterol synthesis, plasmalogen synthesis, amino acid metabolism, and purine metabolism. The oxidative enzymes in peroxisomes protect the cell from metabolic production of hydrogen peroxide by breaking H2O2 down into water and oxygen.

Biochemistry of the Peroxisome @ Peroxisome.org : Beta oxidation : Auxiliary b-oxidation : a-oxidation : Glycerolipid synthesis : Cholesterol biosynthesis : Amino acid metabolism : Polyamine degradation : Purine metabolism : Bile acid synthesis : Biochemistry of the peroxisome membrane : Peroxisomal matrix enzymes : Peroxisomal single enzyme disorders

Defects in these peroxisomal processes manifest in numerous genetic disorders, which can be divided into two categories: disorders resulting from a defect in a single peroxisomal enzyme, and disorders which result from a deficiency in the biogenesis of the peroxisome, affecting all of the metabolic pathways of the peroxisome. Disorders related to the peroxisome can result from mutations in any of at least 13 genes, known as PEX genes. The products of these genes are known as peroxins, and all function in some aspect of peroxisome biogenesis.

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