UniprotKB/SwissProt ID: RN19A_HUMAN (Q9NV58)
Gene Name: RNF19A
Synonyms: RNF19
Organism: Homo sapiens (Human).
Function: 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.
Other Modifications: View all modification sites in dbPTM
Protein Subcellular Localization: Membrane; Multi-pass membrane protein (Potential). Cytoplasm, cytoskeleton, microtubule organizing center, centrosome. Note=Present in the hyaline inclusion bodies specifically found in motor neurons from amyotrophic lateral sclerosis patients. Present i
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