Gene Ontology annotation based on UniPathway vocabulary mapping
Gene Ontology annotation based on UniProtKB/Swiss-Prot Subcellular Location vocabulary mapping
Combined Automated Annotation using Multiple IEA Methods
RNF170 protein, an endoplasmic reticulum membrane ubiquitin ligase, mediates inositol 1,4,5-trisphosphate receptor ubiquitination and degradation.
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RNF170 is an ER-membrane ubiquitin ligase that, constitutively associated with the ERLIN1/ERLIN2 complex, mediates ubiquitination and ERAD-dependent degradation of activated inositol 1,4,5-trisphosphate receptor (ITPR1/IP3R).
A proteome-scale map of the human interactome network.
E3 ubiquitin ligase RNF170 inhibits innate immune responses by targeting and degrading TLR3 in murine cells.
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RNF170 binds TLR3 and catalyzes K48-linked polyubiquitination of Lys-766 in the TLR3 TIR domain, promoting proteasomal degradation of TLR3 and selectively inhibiting TLR3-triggered innate immune responses; RNF170 acts at the ER membrane and its ligase activity requires RING residues Cys-102/His-104.
A reference map of the human binary protein interactome.
Bi-allelic variants in RNF170 are associated with hereditary spastic paraplegia.
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Bi-allelic (recessive) RNF170 variants cause autosomal recessive hereditary spastic paraplegia (SPG85) in four unrelated families; functional studies in patient fibroblasts, mutant SH-SY5Y cells and zebrafish knockdown link the loss of RNF170-mediated ITPR1/IP3R degradation (ERAD) and altered ER calcium signaling to the disease.
ERLIN1/2 scaffolds bridge TMUB1 and RNF170 and restrict cholesterol esterification to regulate the secretory pathway.
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ERLIN1/2 ring-like SPFH-domain scaffolds bind a conserved luminal N-terminal motif shared by RNF170 and the full-length isoform of TMUB1, bridging the two proteins in cholesterol-rich ER nanodomains; variants that preclude these interactions have been linked to hereditary spastic paraplegia, and ERLIN scaffolds limit cholesterol esterification to favour ER-to-Golgi cholesterol transport and regulate the secretory pathway.