E3 ubiquitin-protein ligase that regulates selective mitochondrial autophagy by mediating 'Lys-63'-linked polyubiquitination of BNIP1 . Acts in the endoplasmic reticulum (ER)-associated degradation (ERAD) pathway, which targets misfolded proteins that accumulate in the endoplasmic reticulum (ER) for ubiquitination and subsequent proteasome-mediated degradation . Protects cells from ER stress-induced apoptosis . Responsible for the cotranslational ubiquitination and degradation of CFTR in the ERAD pathway . Also acts as a regulator of the innate antiviral response by catalyzing 'Lys-27'-linked polyubiquitination of CGAS at 'Lys-173' and 'Lys-384', thereby promoting CGAS cyclic GMP-AMP synthase activity . Preferentially associates with the E2 enzymes UBE2J1 and UBE2J2 .
Subcellular locations: Mitochondrion outer membrane, Endoplasmic reticulum membrane
Ubiquitously expressed.