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Details on Person UniProt:O15541 RNF113A

Class:IdReferenceGeneProduct:67894
_chainChangeLoginitiator methionine:1 added on Fri February 6 2015;chain:2-343 added on Fri February 6 2015;initiator methionine:1 for 67894 removed on Fri Nov 03 2023;initiator methionine: for 67894 added on Fri Nov 03 2023;initiator methionine: for 67894 removed on Fri Aug 15 2025;initiator methionine:1 for 67894 added on Fri Aug 15 2025
_displayNameUniProt:O15541 RNF113A
_timestamp2025-08-15 21:33:13
chaininitiator methionine:1
chain:2-343
checksumF76E28037A6FF78B
commentFUNCTION Required for pre-mRNA splicing as component of the spliceosome (PubMed:29360106, PubMed:29361316). As a component of the minor spliceosome, involved in the splicing of U12-type introns in pre-mRNAs (Probable). E3 ubiquitin-protein ligase that catalyzes the transfer of ubiquitin onto target proteins (PubMed:28978524, PubMed:29144457). Catalyzes polyubiquitination of SNRNP200/BRR2 with non-canonical 'Lys-63'-linked polyubiquitin chains (PubMed:29144457). Plays a role in DNA repair via its role in the synthesis of 'Lys-63'-linked polyubiquitin chains that recruit ALKBH3 and the ASCC complex to sites of DNA damage by alkylating agents (PubMed:29144457). Ubiquitinates CXCR4, leading to its degradation, and thereby contributes to the termination of CXCR4 signaling (PubMed:28978524).CATALYTIC ACTIVITY S-ubiquitinyl-[E2 ubiquitin-conjugating enzyme]-L-cysteine + [acceptor protein]-L-lysine = [E2 ubiquitin-conjugating enzyme]-L-cysteine + N(6)-ubiquitinyl-[acceptor protein]-L-lysine.PATHWAY Protein modification; protein ubiquitination.SUBUNIT Component of pre-catalytic and catalytic spliceosome complexes (PubMed:22365833, PubMed:29360106, PubMed:29361316). Interacts (via N-terminus) with the spliceosome subunit SNRNP200/BRR2 (PubMed:29144457). Component of the minor spliceosome, which splices U12-type introns. Within this complex, interacts with SCNM1 and CRIPT (PubMed:33509932).INTERACTION Colocalizes with ASCC2 in nuclear foci after DNA damage by alkylating agents. In the absence of DNA damage, colocalizes with the spliceosome components SNRNP200/BRR2 and PRPF8 in nuclear speckles.TISSUE SPECIFICITY Ubiquitous.DISEASE The disease is caused by variants affecting the gene represented in this entry.
descriptionrecommendedName: E3 ubiquitin-protein ligase RNF113A ecNumber evidence="6 7"2.3.2.27 alternativeName: fullName evidence="13"Cwc24 homolog alternativeName: RING finger protein 113A alternativeName: fullName evidence="12"Zinc finger protein 183
geneNameRNF113A
RNF113
ZNF183
identifierO15541
isSequenceChangedFALSE
keyword3D-structure
Acetylation
DNA damage
DNA repair
Metal-binding
mRNA processing
mRNA splicing
Nucleus
Phosphoprotein
Proteomics identification
Reference proteome
Spliceosome
Transferase
Ubl conjugation pathway
Zinc
Zinc-finger
modified[InstanceEdit:9836292] Weiser, Joel, 2023-05-25
[InstanceEdit:9852000] Weiser, Joel, 2023-11-03
[InstanceEdit:9917590] Weiser, Joel, 2024-08-09
[InstanceEdit:9926675] Weiser, Joel, 2024-11-03
[InstanceEdit:9963647] Weiser, Joel, 2025-08-15
nameRNF113A
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:8993257] ENSEMBL:ENSG00000125352 RNF113A [Homo sapiens]
secondaryIdentifierR113A_HUMAN
B2RBR7
sequenceLength343
species[Species:48887] Homo sapiens
(referenceEntity)[EntityWithAccessionedSequence:9769563] RNF113A [nucleoplasm] [Homo sapiens]
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