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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.
Gene Name: | ring finger protein 19A, RBR E3 ubiquitin protein ligase |
Synonyms: | RNF19A, DORFIN, Double ring-finger protein, RING finger protein 19 isoform, RNF19, Ring finger protein 19, p38, p38 protein, RING finger protein 19A |
Target Sequences: | NM_015435 NP_056250.3 Q9NV58 |
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