Anti-Peroxin 1 / PEX1

Adding 0.2 ml of distilled water will yield a concentration of 500 ug/ml. Peroxin 1 antibody detects Peroxisomal biogenesis factor 1, encoded by the PEX1 gene on chromosome 7q21.2. Peroxin 1 antibody is commonly used in studies of peroxisome biology, metabolism, and genetic disorders. Peroxin 1 is a large AAA ATPase that is essential for peroxisome biogenesis and maintenance. It functions in the recycling of the peroxisomal import receptor PEX5, thereby supporting import of matrix proteins into peroxisomes. This process ensures that enzymes required for beta-oxidation of fatty acids, plasmalogen synthesis, and detoxification of reactive oxygen species are correctly localized.Structurally, Peroxin 1 is a ~147 kDa protein containing two AAA ATPase domains that hydrolyze ATP to drive conformational changes. These domains form a hexameric ring structure that provides mechanical force for receptor recycling. PEX1 localizes to the peroxisomal membrane, where it cooperates with PEX6 to dislocate PEX5 after cargo delivery. Mutations in PEX1 disrupt this process, leading to defective peroxisome assembly.Functionally, Peroxin 1 supports peroxisomal protein import, organelle maintenance, and lipid metabolism. It plays a vital role in processing very long-chain fatty acids, bile acid intermediates, and ether phospholipids. Researchers use Peroxin 1 antibody to study peroxisome biogenesis, metabolic regulation, and genetic disease mechanisms.Clinically, mutations in PEX1 are the most common cause of Zellweger spectrum disorders, a group of peroxisome biogenesis disorders characterized by developmental delay, liver dysfunction, and neurodegeneration. Severity ranges from lethal neonatal disease to milder phenotypes. Understanding PEX1 function is critical for developing therapies for peroxisomal disorders. NSJ Bioreagents supplies Peroxin 1 antibody for research in peroxisome biology and metabolic diseases.Experimentally, Peroxin 1 antibody is used in western blotting to detect the ~147 kDa protein, in immunofluorescence microscopy to visualize peroxisomal localization, and in immunohistochemistry to study tissue-specific expression. Co-immunoprecipitation with Peroxin 1 antibody helps identify interaction partners, including PEX6 and PEX5.