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Peroxisome biogenesis factor 2 (PEX2) is an integral membrane protein of the peroxisome required for the assembly and maintenance of functional peroxisomes. It acts as an E3 ubiquitin-protein ligase that is crucial for the retrotranslocation and recycling of the peroxisomal targeting signal receptor PEX5, enabling import of peroxisomal matrix proteins. PEX2 forms part of a retrotranslocation channel with PEX10 and PEX12, facilitating mono-ubiquitination of PEX5 and peroxisome organization. Mutations in PEX2 cause a spectrum of peroxisome biogenesis disorders including Zellweger syndrome, neonatal adrenoleukodystrophy, and infantile Refsum disease, leading to severe multi-system consequences due to defective peroxisome assembly and function[1][2][4][6][7].
Drugs targeting this pathway could modulate the import/export of peroxisomal proteins, restore peroxisome assembly, or regulate cellular responses to peroxisomal dysfunction, but no direct interacting drugs are documented in available sources[1][2][6][7].
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