功能E3 ubiquitin-protein ligase which accepts ubiquitin from E2 ubiquitin-conjugating enzymes UBE2L3 and UBE2L6 in the form of a thioester and then directly transfers the ubiquitin to targeted substrates, such as SNCAIP or CASR. Specifically ubiquitinates pathogenic SOD1 variants, which leads to their proteasomal degradation and to neuronal protection.
组织特异性Widely expressed, with highest levels in heart. Ubiquitously expressed in the central nervous system.
通路Protein modification; protein ubiquitination.
序列相似性Belongs to the RBR family. RNF19 subfamily. Contains 1 IBR-type zinc finger. Contains 2 RING-type zinc fingers.
翻译后修饰Phosphorylated upon DNA damage, probably by ATM or ATR.
细胞定位Membrane. Cytoplasm > cytoskeleton > centrosome. Present in the hyaline inclusion bodies specifically found in motor neurons from amyotrophic lateral sclerosis patients. Present in the Lewy bodies specifically found in neurons from Parkinson disease patients.