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Peroxisomes owe their name to hydrogen peroxide generating and scavenging activities. They perform key roles in lipid metabolism and the reduction of reactive oxygen species. [4] Peroxisomes are involved in the catabolism of very long chain fatty acids, branched chain fatty acids, bile acid intermediates (in the liver), D-amino acids, and ...
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A peroxisome is a type of microbody that functions to help the body break down large molecules and detoxify hazardous substances. It contains enzymes like oxidase, react hydrogen peroxide as a byproduct of its enzymatic reactions. Within the peroxisome, hydrogen peroxide can then be converted to water by enzymes like catalase and peroxidase.
Peroxisomal disorders represent a class of medical conditions caused by defects in peroxisome functions. [1] This may be due to defects in single enzymes [2] important for peroxisome function or in peroxins, proteins encoded by PEX genes that are critical for normal peroxisome assembly and biogenesis.
Oxidase is a general name for enzymes that catalyze oxidations in which molecular oxygen is the electron acceptor but oxygen atoms do not appear in the oxidized product. Often, oxygen is reduced to either water (cytochrome oxidase of the mitochondrial electron transfer chain) or hydrogen peroxide (dehydrogenation of fatty acyl-CoA in peroxisomes).
Peroxisome proliferator-activated receptor gamma coactivator 1-alpha (PGC-1α) is a protein that in humans is encoded by the PPARGC1A gene. [4] PPARGC1A is also known as human accelerated region 20 . It may, therefore, have played a key role in differentiating humans from apes. [5] PGC-1α is the master regulator of mitochondrial biogenesis.
Unlike the peroxisome biogenesis disorders, other aspects of peroxisome assembly in RCDP2 and RCDP3 patients are normal as is their ability to metabolize very long chain fatty acids. Individuals with severe plasmalogen deficiencies frequently show abnormal neurological development, skeletal malformation, impaired respiration, and cataracts.
In eukaryotes, catalase is usually located in a cellular organelle called the peroxisome. [30] Peroxisomes in plant cells are involved in photorespiration (the use of oxygen and production of carbon dioxide) and symbiotic nitrogen fixation (the breaking apart of diatomic nitrogen (N 2) to reactive nitrogen atoms). Hydrogen peroxide is used as a ...