peroxin14

Peroxin14, also known as PEX14, is a peroxisomal membrane protein that plays a critical role in the biogenesis and function of peroxisomes, which are cellular organelles involved in lipid metabolism and detoxification processes. It is a component of the peroxisomal import machinery responsible for facilitating the import of matrix proteins into peroxisomes.

PEX14 is highly conserved across eukaryotic species, reflecting its essential function. It is primarily localized to the outer membrane of peroxisomes, where it forms part of the peroxisomal translocon complex. PEX14 interacts with other peroxins, such as PEX13 and PEX5, to mediate the docking and translocation of proteins bearing peroxisomal targeting signals (PTS). Specifically, PEX5 serves as a receptor for PTS1- and PTS2-containing proteins, and PEX14 acts as a docking site for PEX5-cargo complexes, enabling their entry into the peroxisomal matrix.

Mutations in PEX14 are associated with peroxisome biogenesis disorders, a subset of peroxisomal biogenesis defects characterized by defective peroxisome formation and function. These disorders can lead to severe neurodevelopmental and metabolic abnormalities, highlighting the importance of PEX14 in cellular homeostasis.

Research on PEX14 continues to focus on its structure-function relationships, mechanisms of protein import, and its potential as a target for therapeutic intervention in peroxisomal diseases. Understanding the precise molecular interactions of PEX14 enhances insights into peroxisomal dynamics and the maintenance of cellular metabolism.