Endoplasmic reticulum lipid raft-associated protein 1, Protein KE04, Stomatin-prohibitin-flotillin-HflC/K domain-containing protein 1, SPFH domain-containing protein 1 | C10orf69, KE04, KEO4, SPFH1
Primary ID
O75477
Links
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Type
protein
Relations
1
Function
Component of the ERLIN1/ERLIN2 complex which mediates the endoplasmic reticulum-associated degradation (ERAD) of inositol 1,4,5-trisphosphate receptors (IP3Rs). Involved in regulation of cellular cholesterol homeostasis by regulation the SREBP signaling pathway. Binds cholesterol and may promote ER retention of the SCAP-SREBF complex (PubMed:24217618).
Here we report that the ER membrane protein SPFH1 and its homolog SPFH2 form a heteromeric approximately 2 MDa complex that binds to IP(3)R tetramers immediately after their activation and is required for their processing. The complex is ring-shaped (diameter approximately 250A(),) and RNA interference-mediated depletion of SPFH1 and SPFH2 blocks IP(3)R polyubiquitination and degradation.