PEX1PEX6

Pex1Pex6 refers to a protein complex composed of two AAA-ATPases, Pex1 and Pex6, which are involved in peroxisome biogenesis and function. These ATPases form a heteromeric complex integral to the import of peroxisomal matrix proteins and the maintenance of peroxisome integrity. The Pex1Pex6 complex is highly conserved across eukaryotic species, highlighting its essential role in cellular metabolism.

Pex1 and Pex6 work together by harnessing the energy from ATP hydrolysis to facilitate the translocation of proteins into the peroxisomal matrix and to recycle peroxisomal import receptors. Structurally, both proteins contain AAA+ ATPase domains that enable their oligomerization and the formation of a ring-shaped complex. This assembly interacts with other peroxins and components of the peroxisomal import machinery.

Defects or mutations in Pex1 or Pex6 are associated with peroxisomal biogenesis disorders, most notably Zellweger spectrum disorders, which are characterized by defective peroxisome formation and dysfunctional lipid metabolism. These conditions highlight the importance of the Pex1Pex6 complex in cellular homeostasis.

Research continues to elucidate the detailed mechanisms of Pex1Pex6 function and regulation. Understanding these processes provides insights into peroxisomal disorders and may inform potential therapeutic strategies. The complex’s role in maintaining peroxisome dynamics is crucial for lipid metabolism, reactive oxygen species detoxification, and other vital cellular processes.