Pex13Pex14

PEX13 and PEX14 are two proteins that play crucial roles in the process of peroxisome biogenesis, a cellular function responsible for the formation and maintenance of peroxisomes. Peroxisomes are organelles found in the cells of eukaryotes, involved in various metabolic processes such as the breakdown of fatty acids and the detoxification of hydrogen peroxide.

PEX13, also known as peroxisomal biogenesis factor 13, is an integral membrane protein located in the peroxisomal membrane. It functions as a docking protein, facilitating the translocation of peroxisomal matrix proteins into the peroxisomal lumen. PEX13 interacts with other peroxins, such as PEX14 and PEX19, to form a complex that is essential for the import of peroxisomal matrix proteins.

PEX14, or peroxisomal biogenesis factor 14, is another integral membrane protein found in the peroxisomal membrane. It acts as a receptor for the peroxisomal targeting signal 1 (PTS1), which is a sequence found at the C-terminus of many peroxisomal matrix proteins. PEX14 forms a stable complex with PEX13 and PEX5, another peroxin involved in the import of peroxisomal matrix proteins. This complex is crucial for the efficient translocation of PTS1-containing proteins into the peroxisomal matrix.

Mutations in the genes encoding PEX13 and PEX14 have been linked to various peroxisomal biogenesis disorders, collectively known as Zellweger syndrome spectrum disorders. These disorders are characterized by the impaired formation of peroxisomes, leading to a range of clinical symptoms, including developmental delays, neurological abnormalities, and liver dysfunction. Understanding the functions of PEX13 and PEX14 is therefore essential for the development of potential therapies for these debilitating conditions.