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Details on Person UniProt:O75643 SNRNP200

Class:IdReferenceGeneProduct:71884
_chainChangeLogchain:1-2136 added on Fri February 6 2015
_displayNameUniProt:O75643 SNRNP200
_timestamp2025-02-21 20:05:07
chainchain:1-2136
checksum3F3811BDCCC9DDB7
commentFUNCTION Catalyzes the ATP-dependent unwinding of U4/U6 RNA duplices, an essential step in the assembly of a catalytically active spliceosome (PubMed:35241646). Plays a role in pre-mRNA splicing as a core component of precatalytic, catalytic and postcatalytic spliceosomal complexes (PubMed:28502770, PubMed:28781166, PubMed:29301961, PubMed:29360106, PubMed:29361316, PubMed:30315277, PubMed:30705154, PubMed:30728453). As a component of the minor spliceosome, involved in the splicing of U12-type introns in pre-mRNAs (Probable). Involved in spliceosome assembly, activation and disassembly. Mediates changes in the dynamic network of RNA-RNA interactions in the spliceosome.CATALYTIC ACTIVITY ATP + H2O = ADP + phosphate + H(+)SUBUNIT Component of a core complex containing at least PRPF8, SNRNP200, EFTUD2 and SNRNP40. Component of the U5 snRNP and U4/U6-U5 tri-snRNP complexes, building blocks of the spliceosome. Component of the U4/U6-U5 tri-snRNP complex composed of the U4, U6 and U5 snRNAs and at least PRPF3, PRPF4, PRPF6, PRPF8, PRPF31, SNRNP200, TXNL4A, SNRNP40, DDX23, CD2BP2, PPIH, SNU13, EFTUD2, SART1 and USP39 (PubMed:16723661, PubMed:26912367, PubMed:8670905). Component of precatalytic, catalytic and postcatalytic spliceosomal complexes (PubMed:11991638, PubMed:28502770, PubMed:28781166, PubMed:29301961, PubMed:29360106, PubMed:29361316, PubMed:30315277, PubMed:30705154, PubMed:30728453, PubMed:35241646). Component of the minor spliceosome, which splices U12-type introns (PubMed:33509932). Interacts with C9orf78; the interaction is direct and mutually exclusive with its interaction with WBP4 (PubMed:35241646). Interacts with WBP4; the interaction is mutually exclusive with its interaction with C9orf78 (PubMed:35241646). Interacts with PRPF8 (PubMed:35241646). Interacts with TSSC4; the interaction is direct, excludes recruitment of C9ORF78 and WBP4 to SNRNP200 and negatively regulates its RNA helicase activity (PubMed:35188580).INTERACTION Widely expressed.DOMAIN Contains two helicase domains. The N-terminal helicase domain has catalytic activity by itself, contrary to the C-terminal helicase domain that may have a regulatory role and enhance the activity of the first helicase domain.DISEASE The disease is caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the helicase family. SKI2 subfamily.SEQUENCE CAUTION Truncated N-terminus.
descriptionrecommendedName: U5 small nuclear ribonucleoprotein 200 kDa helicase ecNumber evidence="14 28"3.6.4.13 alternativeName: Activating signal cointegrator 1 complex subunit 3-like 1 alternativeName: BRR2 homolog alternativeName: U5 snRNP-specific 200 kDa protein shortName: U5-200KD
geneNameSNRNP200
ASCC3L1
BRR2
HELIC2
KIAA0788
identifierO75643
isSequenceChangedFALSE
keyword3D-structure
Acetylation
Alternative splicing
ATP-binding
Coiled coil
Direct protein sequencing
Disease variant
Helicase
Hydrolase
Isopeptide bond
mRNA processing
mRNA splicing
Nucleotide-binding
Nucleus
Phosphoprotein
Proteomics identification
Reference proteome
Repeat
Retinitis pigmentosa
Spliceosome
Ubl conjugation
modified[InstanceEdit:9836292] Weiser, Joel, 2023-05-25
[InstanceEdit:9841277] Weiser, Joel
[InstanceEdit:9852000] Weiser, Joel, 2023-11-03
[InstanceEdit:9917590] Weiser, Joel, 2024-08-09
[InstanceEdit:9926675] Weiser, Joel, 2024-11-03
[InstanceEdit:9939033] Weiser, Joel, 2025-02-21
nameSNRNP200
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:9002121] ENSEMBL:ENSG00000144028 SNRNP200 [Homo sapiens]
secondaryIdentifierU520_HUMAN
O94884
Q6NZY0
Q6PX59
Q8NBE6
Q96IF2
Q9H7S0
sequenceLength2136
species[Species:48887] Homo sapiens
(isoformParent)[ReferenceIsoform:253368] UniProt:O75643-2 SNRNP200 [Homo sapiens]
[ReferenceIsoform:401881] UniProt:O75643-1 SNRNP200 [Homo sapiens]
(referenceEntity)[EntityWithAccessionedSequence:71885] SNRNP200 [nucleoplasm] [Homo sapiens]
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