Target intelligence / Profile preview

Peroxisomal membrane protein 14 (PEX14)

Target
PEX14
Molecular classification
Other (Peroxisome membrane protein, Import machinery component)
01

Overview

Peroxisomal membrane protein 14 (PEX14) is an essential integral membrane protein of peroxisomes, crucial for peroxisomal protein import and biogenesis. It serves as a central docking site for soluble receptor proteins, primarily PEX5 and PEX7, which mediate the delivery of matrix proteins carrying peroxisomal targeting signals (PTS1 and PTS2)[1][2][3][4][6]. The C-terminus of PEX14 is exposed to the cytosol, facilitating interaction with cytosolic trafficking factors. PEX14 also has a role as a transcriptional corepressor by interacting with histone deacetylases[1]. Mutations in PEX14 can cause peroxisome biogenesis disorders, such as Zellweger syndrome, characterized by severe neurological and metabolic abnormalities[1][6]. Structurally, PEX14 contains domains necessary for membrane integration and specific interaction motifs that enable competitive binding with PEX5 and PEX19, key proteins in peroxisome assembly and maintenance[3][4][7]. PEX14 is indispensable for both the import of new proteins and for pexophagy, thus balancing peroxisome formation and turnover[2][5]. Interactions with other peroxins include PEX13 and potentially PEX19, emphasizing its central role in peroxisome dynamics and cellular metabolism[1][3][4].

Other names
PEX14Peroxisomal biogenesis factor 14Pex14pPeroxisomal membrane anchor protein PEX14PTS1 receptor-docking proteinPeroxin-14NAPP2PBD13AdJ734G22.2NF-E2 associated polypeptide 2
02

Biological functions

Peroxisomal protein importPeroxisome biogenesisDocking of receptor proteins (Pex5, Pex7)Transcriptional corepression (interacts with histone deacetylase)Peroxisome motilityPexophagy (selective degradation of peroxisomes)
03

Disease associations

Peroxisome biogenesis disorders (PBDs), notably Zellweger syndrome spectrum

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