PEX26 Human
Description
Product Specs
Product Science Overview
The PEX26 gene belongs to the peroxin-26 gene family and is located on chromosome 22. The gene encodes a protein that is approximately 305 amino acids in length. The PEX26 protein is characterized by its ability to anchor other peroxisomal proteins, specifically PEX1 and PEX6, to the peroxisome membrane. This anchoring is essential for the formation of the PEX1-PEX6 AAA ATPase complex, which mediates the extraction of the PEX5 receptor from the peroxisomal membrane .
PEX26 is required for the import of proteins into peroxisomes. It facilitates the docking of PEX1 and PEX6, forming a heteromeric AAA ATPase complex necessary for the import process. This complex plays a pivotal role in the recycling of the PEX5 receptor, which is responsible for transporting peroxisomal matrix proteins into the organelle .
Mutations in the PEX26 gene are associated with peroxisome biogenesis disorders (PBDs), specifically complementation group 8 (PBD-CG8). PBDs are a group of genetic disorders that result from defects in peroxisomal protein import, leading to a failure in peroxisome formation and function. These disorders include Zellweger syndrome, neonatal adrenoleukodystrophy, infantile Refsum disease, and classical rhizomelic chondrodysplasia punctata .
Recombinant human PEX26 is produced using recombinant DNA technology, where the PEX26 gene is cloned and expressed in a suitable host system, such as E. coli. The recombinant protein is often tagged with a His-tag to facilitate purification. This recombinant form is used in various research applications to study the function and pathology of PEX26 and related peroxisomal disorders .
Research on PEX26 has provided significant insights into the molecular mechanisms of peroxisomal biogenesis and the pathogenesis of PBDs. Understanding the role of PEX26 in peroxisome assembly and function has implications for developing therapeutic strategies for PBDs. Recombinant PEX26 is also used in biochemical assays to study protein-protein interactions and the dynamics of peroxisomal protein import .
