Pex1 Protein Peroxisome Biogenesis Factor 1 is an important component in the neurobiology of neurodegenerative diseases. This page provides detailed information about its structure, function, and role in disease processes.
PEX1 (Peroxisome Biogenesis Factor 1) is a member of the AAA (ATPases Associated with diverse cellular Activities) ATPase family essential for peroxisome biogenesis [1].
PEX1 PROTEIN is a gene/protein encoding a key neuronal protein involved in synaptic function, signal transduction, and cellular homeostasis. Dysfunction of PEX1 PROTEIN is associated with neurodegenerative diseases including Alzheimer's disease, Parkinson's disease, and related disorders.
| Property | Value |
|---|---|
| Protein Name | Pex1p (Peroxin-1) |
| Gene | PEX1 |
| UniProt ID | P50579 |
| PDB ID | 6A6P (yeast homolog) |
| Molecular Weight | ~144 kDa |
| Subcellular Localization | Peroxisomal matrix |
PEX1 contains two AAA ATPase domains (ATP-binding cassettes) and belongs to the AAA+ protein family. The protein forms a hexameric ring structure. The ATPase activity is essential for its function in receptor recycling [2].
PEX1 forms a complex with PEX6 and plays two critical roles:
Receptor Recycling: The PEX1-PEX6 complex uses ATP hydrolysis to extract the peroxisomal targeting receptor PEX5 from the peroxisomal membrane after protein import, recycling it back to the cytosol for another round of import [3].
Peroxisome Assembly: Essential for proper peroxisome biogenesis and maintenance.
PEX1 is the most commonly mutated gene in Zellweger spectrum disorders. Mutations cause [4]:
Peroxisomal deficiency leads to:
No FDA-approved drugs target PEX1 directly. Current approaches include [5]:
The study of Pex1 Protein Peroxisome Biogenesis Factor 1 has evolved significantly over the past decades. Research in this area has revealed important insights into the underlying mechanisms of neurodegeneration and continues to drive therapeutic development.
Historical context and key discoveries in this field have shaped our current understanding and will continue to guide future research directions.
Page updated: 2026-03-05