PEX19
Description
The PEX19 (peroxisomal biogenesis factor 19) is a protein-coding gene located on chromosome 1.
Peroxisomal biogenesis factor 19 is a protein that in humans is encoded by the PEX19 gene.
PEX19 is essential for the early stages of peroxisome formation. It acts as both a chaperone in the cytoplasm and an import receptor for peroxisomal membrane proteins (PMPs). PEX19 binds to newly synthesized PMPs, stabilizing them in the cytoplasm by interacting with their hydrophobic transmembrane domains. It then guides these PMPs to the peroxisome membrane by attaching to the integral membrane protein PEX3. Additionally, PEX19 prevents the tumor suppressor CDKN2A from entering the nucleus and interacting with MDM2, thereby preventing the degradation of TP53.
PEX19 is also known as D1S2223E, HK33, PBD12A, PMP1, PMPI, PXF, PXMP1.
Associated Diseases
- Neonatal adrenoleukodystrophy
- Infantile Refsum disease
- Peroxisome biogenesis disorder 12A (Zellweger)
- Zellweger syndrome
