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Details on Person UniProt:O75643 SNRNP200
| Class:Id | ReferenceGeneProduct:71884 |
|---|---|
| _chainChangeLog | chain:1-2136 added on Fri February 6 2015 |
| _displayName | UniProt:O75643 SNRNP200 |
| _timestamp | 2025-02-21 20:05:07 |
| chain | chain:1-2136 |
| checksum | 3F3811BDCCC9DDB7 |
| comment | FUNCTION 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. |
| description | recommendedName: 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 |
| geneName | SNRNP200 ASCC3L1 BRR2 HELIC2 KIAA0788 |
| identifier | O75643 |
| isSequenceChanged | FALSE |
| keyword | 3D-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 |
| name | SNRNP200 |
| referenceDatabase | [ReferenceDatabase:2] UniProt |
| referenceGene | [ReferenceDNASequence:9002121] ENSEMBL:ENSG00000144028 SNRNP200 [Homo sapiens] |
| secondaryIdentifier | U520_HUMAN O94884 Q6NZY0 Q6PX59 Q8NBE6 Q96IF2 Q9H7S0 |
| sequenceLength | 2136 |
| 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] |
| [Change default viewing format] | |
No pathways have been reviewed or authored by UniProt:O75643 SNRNP200 (71884)
