InterPro domain: IPR038896

General Information

  • Identifier IPR038896
  • Description E3 ubiquitin-protein ligase RNF170
  • Number of genes 240
  • Gene duplication stats Loading...
  • Associated GO terms GO:0061630  

Abstract

RNF170 is an endoplasmic reticulum membrane ubiquitin ligase that contributes to the ubiquitination of activated inositol 1,4,5-trisphosphate (IP3) receptors [ 1 ]. When point mutated (arginine to cysteine at position 199), it causes autosomal dominant sensory ataxia (ADSA), a disease characterised by neurodegeneration in the posterior columns of the spinal cord [ 2 ].


1. RNF170 protein, an endoplasmic reticulum membrane ubiquitin ligase, mediates inositol 1,4,5-trisphosphate receptor ubiquitination and degradation. J. Biol. Chem. 286, 24426-33
2. A Point Mutation in the Ubiquitin Ligase RNF170 That Causes Autosomal Dominant Sensory Ataxia Destabilizes the Protein and Impairs Inositol 1,4,5-Trisphosphate Receptor-mediated Ca2+ Signaling. J. Biol. Chem. 290, 13948-57

Species distribution

Gene table