Rabbit Polyclonal PEX1 Antibody

The PEX1 antibody is a crucial tool in studying peroxisome biogenesis and related disorders. PEX1. a member of the AAA-ATPase protein family, is essential for peroxisome assembly and function. It forms a heterohexameric complex with PEX6. facilitating the recycling of peroxisomal matrix protein import receptors (e.g., PEX5) from the peroxisomal membrane. Mutations in the PEX1 gene are the most common cause of Zellweger spectrum disorders (ZSDs), a group of autosomal recessive peroxisome biogenesis defects characterized by severe neurological, hepatic, and metabolic abnormalities. PEX1 antibodies are widely used in research to detect PEX1 protein expression, assess its subcellular localization via immunofluorescence, and investigate pathogenic mechanisms in ZSDs. These antibodies also aid in diagnostic workflows to confirm PEX1 deficiency in patient-derived cells. Additionally, studies utilizing PEX1 antibodies have advanced understanding of peroxisome dynamics, protein-protein interactions, and therapeutic approaches, such as pharmacological chaperones to rescue mutant PEX1 function. Their specificity and reliability make PEX1 antibodies indispensable for both basic peroxisome biology and translational research in rare metabolic diseases.