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Peroxisomal Fatty Acid Oxidation Pathway


Peroxisomes are small organelles ubiquitous to animal and plant cells that give their name to a class of proteins, the PPARs or peroxisome proliferator-activated receptors, that are now widely studied because of their relation to metabolic diseases including diabetes and obesity. The terminology is based on the observation that these nuclear receptor proteins control the levels of peroxisomal proteins, but what are peroxisomes? Remarkably, very little was known about these organelles until recently. Peroxisomes are involved in fatty acid oxidation which is regulated by PPARs and hence, the link to diabetes. They also play a role in amino acid catabolism. Recent studies have established the clear link between peroxisomes and mitochondria, not just in fatty acid oxidation, but both have complex and pleomorphic morphology. Peroxisomes undergo fusion and fission events utilizing many of the same proteins driving these processes in mitochondria.

Peroxisomes can be thought of as processing centers to convert complex fatty acids such as branched chain and omega fatty acids into forms that can be further metabolized by the mitochondrion. The sequence of reactions is shown simplistically in the peroxisomal fatty acid oxidation pathway diagram. An important difference between fatty acid oxidation in mitochondria and peroxisomes is that electrons released in the former are captured as NADH for use in energy production, while those released within the peroxisome are removed by catalase to produce hydrogen peroxide.

An inherited inability of peroxisomes to modify complex fatty acid species leads to a number of relatively common diseases including Zellwegers syndrome (through altered organelle biogenesis), adreno-leukodystrophy (mutations in ABCD1), Refsum disease (PHYH), non-specific X linked mental retardation (mutations in FACL4) and others.

Selected Readings:

Schrader M & Fahimi HD. The peroxisome: still a mysterious organelle. Histochem Cell Biol. 2008 Apr;129(4):421-40.

Camoes F, et al. Organelle dynamics and dysfunction: A closer link between peroxisomes and mitochondria. J Inherit Metab Dis. 2009 Apr;32(2):163-80.

Thoms S, et al. Organelle interplay in peroxisomal disorders. Trends Mol Med. 2009 Jul;15(7):293-302.

Wanders RJ, et al. Peroxisomes, lipid metabolism and lipotoxicity. Biochim Biophys Acta. 2010 Mar;1801(3):272-80.

Monoclonal Antibodies
Cat. No. Name Reactivity Apps. Amount Price  
MS716 ACAA1 antibody human, rat, mouse WB, ICC, IP, ICE 100 µg $325.00
MS721 Catalase antibody human, bovine, rat ICC, IP, ICE 100 µg $325.00
MS723 ECH1 antibody human, rat ICC, IP, ICE 100 µg $325.00
MS730 ECHD antibody human ICC, IP 100 µg $325.00
MS727 MFE2 antibody human IP 100 µg $325.00 <

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Peroxisomal Fatty Acid Oxidation Pathway Diagram

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