Peroxin 26 Antibody (A-9): sc-376817

---

Peroxisomes are single-membrane bound organelles present in virtually all eukaryotic cells. They are involved in numerous catabolic and anabolic pathways, including beta-oxidation of very long chain fatty acids, metabolism of hydrogen peroxide, plasmalogen biosynthesis and bile acid synthesis. The Peroxin gene family, which includes more than 20 members, is required for peroxisome biogenesis. Peroxin 26, also known as PEX26 (Peroxisome assembly protein 26) is a widely expressed protein that functions to recruit, shuttle and anchor Peroxin 1 and Peroxin 6 to the peroxisome membrane, thus allowing the formation of heteromeric AAA ATPase complexes. Once formed, the ATPase complexes are able to import various proteins, such as catalase, into peroxisomes. Proper import of these peroxisomal proteins is essential for normal development. Defects in the gene encoding Peroxin 26 are the cause of multiple peroxisome-related disorders, including Zellweger syndrome (ZWS), infantile Refsum disease (IRD) and peroxisome biogenesis disorder complementation group 8 (PBD-CG8).