Target intelligence / Profile preview

Peroxisome biogenesis factor 2 (PEX2)

Target
PEX2
Molecular classification
Enzyme (E3 ubiquitin-protein ligase), Integral membrane protein, Peroxin
01

Overview

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].

Other names
Peroxisomal biogenesis factor 2PEX2PAF1PMP3PMP35PXMP3RNF72PAF-1ZWS335 kDa peroxisomal membrane proteinPeroxin-2Peroxisomal membrane protein 3Peroxisome assembly factor 1RING finger protein 72
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Mechanism of action

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].

03

Biological functions

Peroxisome biogenesisImport of peroxisomal matrix proteinsRegulation of peroxisome organizationMono- and polyubiquitination of protein substratesPexophagy (peroxisome turnover)Response to reactive oxygen species
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Disease associations

Zellweger syndrome (Zellweger spectrum disorder)Neonatal adrenoleukodystrophyInfantile Refsum diseaseOther peroxisome biogenesis disorders
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Safety considerations

Broad disruption of PEX2 function could cause severe peroxisomal dysfunction, mimicking Zellweger spectrum disease phenotypes[4].Potential for off-target effects on lipid metabolism and cellular responses to oxidative stress.

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