FEATURES

FAMILY

DESCRIPTION

Also known as RN19A_HUMAN, RNF19A, RNF19. 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. Interacts with UBE2L3 and UBE2L6. Interacts with transcription factor Sp1. Interacts with VCP, CASR, SNCAIP and with some SOD1 variants which cause amyotrophic lateral sclerosis, but not with wild-type SOD1.

EXTERNAL LINKS

uniprot
enzyme classification
PhosphoSitePlus

RNF19A

E3 ubiquitin-protein ligase RNF19A

RNF19A

Molecular Synopsis