Anti-PEX1 / Peroxisome biogenesis factor 1

Adding 0.2 ml of distilled water will yield a concentration of 500 ug/ml. PEX1 antibody detects Peroxisome biogenesis factor 1, an ATPase essential for the assembly and maintenance of functional peroxisomes. Encoded by the PEX1 gene on chromosome 7q21.2, this cytoplasmic AAA-type ATPase facilitates peroxisomal matrix protein import by recycling the peroxisomal targeting receptor PEX5. PEX1 acts in concert with PEX6 to drive the dislocation and recycling of receptor proteins from the peroxisomal membrane back to the cytosol, ensuring continued import of matrix enzymes required for lipid metabolism and detoxification.PEX1 contains two ATPase domains characteristic of the AAA+ protein family and forms a complex with PEX6 that attaches to the peroxisomal membrane via PEX26. This complex functions as an energy-dependent disassembly machine, resetting the import apparatus after cargo delivery. Proper PEX1 activity is essential for peroxisome maintenance and metabolic homeostasis, including beta-oxidation of very long-chain fatty acids and plasmalogen synthesis.The PEX1 antibody is widely used in cell biology, metabolism, and genetic disease research to study peroxisomal biogenesis, import machinery, and metabolic regulation. Western blot analysis identifies a 147 kilodalton band corresponding to PEX1, while immunofluorescence shows punctate cytoplasmic staining consistent with peroxisomal localization. This antibody provides a sensitive tool for investigating peroxisomal function and organelle dynamics.Mutations in PEX1 are the most common cause of Zellweger spectrum disorders, a group of peroxisomal biogenesis disorders leading to severe metabolic dysfunction. Loss of PEX1 disrupts enzyme import, leading to defective peroxisome assembly and accumulation of toxic lipid intermediates. The PEX1 antibody supports studies of peroxisome biology, organelle biogenesis, and genetic metabolic disease. NSJ Bioreagents provides this antibody validated for its applications, ensuring reliability for cellular and metabolic research.