peroxisome

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Peroxisome

An intracellular organelle found in all eukaryotes except the archezoa (original lifeforms). In electron micrographs, peroxisomes appear round with a diameter of 0.1–1.0 micrometer, although there is evidence that in some mammalian tissues peroxisomes form an extensive reticulum (network). They contain more than 50 characterized enzymes and perform many biochemical functions, including detoxification. See Cell organization, Enzyme

Peroxisomes are important for lipid metabolism. In humans, the β-oxidation of fatty acids greater than 18 carbons in length occurs in peroxisomes. In yeast, all fatty acid β-oxidation occurs in peroxisomes. Peroxisomes contain the first two enzymes required for the synthesis of plasmalogens. Peroxisomes also play important roles in cholesterol and bile acid synthesis, purine and polyamine catabolism, and prostaglandin metabolism. In plants, peroxisomes are required for photorespiration. See Lipid metabolism, Photorespiration

A number of recessively inherited peroxisomal disorders have been described and grouped into three categories. Group I is the most severe and is characterized by a general loss of peroxisomal function. Many of the enzymes normally localized to the peroxisome are instead found in the cytosol. Among the diseases found in group I are Zellweger syndrome, neonatal adrenoleukodystrophy, and infantile Refsum disease. Patients with these disorders usually die within the first years after birth and exhibit neurological and hepatic (liver) dysfunction, along with craniofacial dysmorphism (malformation of the cranium and the face). Groups II and III peroxisomal disorders are characterized by a loss of peroxisomal function less severe than in group I.

peroxisome

[pə′räk·sə‚sōm]
(cell and molecular biology)
Any of a subclass of microbodies that contain at least four enzymes involved in the metabolism of hydrogen peroxide.
References in periodicals archive ?
In summary, the observation that supplemental trans-10, cis-12 CLA increased steady-state concentration of ACO A-I mRNA in HepG2 cells suggests a role for this CLA isomer in the control of peroxisomal oxidation of fatty acids in the liver.
Compatibility of glycinebetaine in rice plants: Evaluation using transgenic rice plants with a gene for peroxisomal betaine aldehyde dehydrogenase from barley.
Modulation of rat liver peroxisomal and microsomal fatty acid oxidation by starvation.
Due the complexities of peroxisomal disorders, the introduction of X-ALD screening programs will require the guidance of NBS specialists and policy makers in regard to feasibility, protocols, risks and benefits (53).
Pharmaceutical company Retrophin (NasdaqGM:RTRX) reported on Wednesday the receipt of first approval from the US Food and Drug Administration (FDA) for Cholbam capsules for treating pediatric and adult patients with bile acid synthesis disorders due to single enzyme defects plus peroxisomal disorders,including Zellweger spectrum disorders.
Part 2 covers small molecule diseases, such as disorders of amino acids, organic acids, neurotransmitters, and mitochondria, while Part 3 reports on work in large molecule diseases, such as defects of glycosylation, lysosomal and peroxisomal function, and leukodystrophies.
Liver peroxisomal fatty acid oxidizing system during aging in control and clofibate-treated mice.
In the present study, we have utilized a combination of betaine and DMSO to amplify the putative promoter region of mouse peroxisomal protein (PeP).
The gene closest to the association signal, PECR (which encodes the enzyme peroxisomal trans-2-enoyl-CoA reductase) is involved in the metabolism of fatty acids, particularly during deprivation when energy expenditure transitions from carbohydrates to fatty acids.
Bian F, Kasumov T, Thomas KR, et al Peroxisomal and mitochondrial oxidation of fatty acids in the heart, assessed from the 13C labeling of malonyl-CoA and the acetyl moiety of citrate.
Serum lipids, hepatic glycerolipid metabolism and peroxisomal fatty acid oxidation in rats fed omega-3 and omega-6 fatty acids.