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Protein that mediates ER-mitochondria trafficking (PERMIT (also known as p17))
- Target
- PERMIT (also known as p17)
- Molecular classification
- Other, Mitochondrial trafficking chaperone protein
01
Overview
Protein that mediates ER-mitochondria trafficking (PERMIT, also known as p17), originally misannotated as "ribosomal protein L29 pseudogene 31" (RPL29P31), is a 17-kDa mitochondrial trafficking chaperone. Unlike typical pseudogenes, PERMIT encodes a fully functional protein responsible for facilitating the transfer of ceramide synthase 1 (CerS1) from the endoplasmic reticulum to mitochondria in response to cellular stress and aging. This process enables localized C18-ceramide synthesis and initiates mitophagy, the selective autophagic removal of damaged mitochondria, thereby maintaining mitochondrial integrity. Loss of PERMIT impairs mitochondrial quality control and accelerates neurodegeneration and sensorimotor decline in experimental models, establishing it as a critical regulator at the intersection of lipid metabolism, mitophagy, and aging biology[7][2][6][4].
- Other names
- p17PERMITProtein that mediates ER-mitochondria trafficking
02
Mechanism of action
Not applicable / none for drugs (the mechanism described is endogenous: p17/PERMIT chaperones Ceramide Synthase 1 to mitochondria in response to stress, facilitating lipid-driven mitophagy[2][7])
03
Biological functions
Mitochondrial quality controlER-mitochondria lipid traffickingMediator of mitophagyStress response
04
Disease associations
Neurodegenerative diseaseAgingBrain injury
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