PEX13

Description

The PEX13 (peroxisomal biogenesis factor 13) is a protein-coding gene located on chromosome 2.

Peroxisomal membrane protein PEX13 is a protein encoded by the PEX13 gene in humans. It is located on chromosome 2, adjacent to KIAA1841. PEX13 interacts with PEX14, PEX5, and PEX19.

PEX13 forms a complex with PEX14, acting as a translocon channel that imports peroxisomal cargo proteins bound to the PEX5 receptor. This complex forms a large import pore, allowing for the passage of proteins. The process begins with the association of the PEX5 receptor and cargo proteins with PEX14 in the cytosol. This leads to the insertion of the receptor into the organelle membrane and the translocation of the cargo into the peroxisome matrix. PEX13 is involved in importing proteins containing PTS1 and PTS2 signals.

PEX13 is also known as NALD, PBD11A, PBD11B, ZWS.

Associated Diseases

• Neonatal adrenoleukodystrophy
• Infantile Refsum disease
• Peroxisome biogenesis disorder 11B
• Peroxisome biogenesis disorder 11A (Zellweger)
• Zellweger syndrome