U5 small nuclear ribonucleoprotein 200 kDa helicase

UniProt O75643 Organism Homo sapiens Gene ASCC3L1, BRR2, HELIC2, KIAA0788, SNRNP200 EC 3.6.4.13

Also known as: ASCC3L1, BRR2, HELIC2, KIAA0788, SNRNP200

Function

Catalyzes the ATP-dependent unwinding of U4/U6 RNA duplices, an essential step in the assembly of a catalytically active spliceosome. Plays a role in pre-mRNA splicing as a core component of precatalytic, catalytic and postcatalytic spliceosomal complexes. 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.

Classification

Family (Pfam): PF21188 BRR2_plug, PF00270 DEAD, PF00271 Helicase_C, PF18149 Helicase_PWI, PF02889 Sec63, PF23445 WHD_SNRNP200
InterPro: Brr2_helicase_PWI, BRR2_plug, C2_domain_sf, DEAD/DEAH_box_helicase_dom, Hel308_SKI2-like, Helicase_ATP-bd, Helicase_C-like, Ig_E-set, P-loop_NTPase, Sec63-dom, WH-like_DNA-bd_sf, WH_DNA-bd_sf, WH_MER3
Functional cluster: S-Adenosylmethionine Synthases & related

Experimental structures · PDB · 79

2Q0Z X-ray 2.00A
3JCR EM 7.00A
4F91 X-ray 2.70A
4F92 X-ray 2.66A
4F93 X-ray 2.92A
4KIT X-ray 3.60A
5O9Z EM 4.50A
5URJ X-ray 2.75A
5URK X-ray 2.95A
5URM X-ray 2.80A
5XJC EM 3.60A
5YZG EM 4.10A
… and 67 more

A predicted model is available from AlphaFold.

Gene Ontology · 22

Disease associations

retinitis pigmentosa 33 MONDO:0012477

Neighborhood · nearest proteins

Nearest neighbours of U5 small nuclear ribonucleoprotein 200 kDa helicase: 3 by sequence/function similarity (left); 3 co-cited in the literature (right).

Related proteins · sequence + function similarity

Co-cited proteins · studied together in the literature

Pre-mRNA-splicing helicase BRR2 1 shared papers
Pre-mRNA-processing-splicing factor 8 13 shared papers
U5 small nuclear ribonucleoprotein TSSC4 1 shared papers
Splicing factor C9orf78 1 shared papers
WW domain-binding protein 4 2 shared papers
U5 small nuclear ribonucleoprotein 40 kDa protein 9 shared papers
116 kDa U5 small nuclear ribonucleoprotein component 10 shared papers
Cell division cycle 5-like protein 7 shared papers
ATP-dependent RNA helicase DHX15 1 shared papers
Pre-mRNA-processing factor 17 5 shared papers
Serine/arginine repetitive matrix protein 2 6 shared papers
RNA-binding motif protein, X-linked 2 3 shared papers

Literature · 42 cited papers

A multi-factor trafficking site on the spliceosome remodeling enzyme BRR2 recruits C9ORF78 to regulate alternative splicing. Nat. Commun. · 2022
The intrinsically disordered TSSC4 protein acts as a helicase inhibitor, placeholder and multi-interaction coordinator during snRNP assembly and recycling. Nucleic Acids Res. · 2022
Structure of the activated human minor spliceosome. Science · 2021
Structures of the human spliceosomes before and after release of the ligated exon. Cell Res. · 2019
A human postcatalytic spliceosome structure reveals essential roles of metazoan factors for exon ligation. Science · 2019
Structures of the human pre-catalytic spliceosome and its precursor spliceosome. Cell Res. · 2018
Structure and Conformational Dynamics of the Human Spliceosomal Bact Complex. Cell · 2018
Structure of the human activated spliceosome in three conformational states. Cell Res. · 2018
Structure of a human catalytic step I spliceosome. Science · 2018
Cryo-EM Structure of a Pre-catalytic Human Spliceosome Primed for Activation. Cell · 2017
An Atomic Structure of the Human Spliceosome. Cell · 2017
Site-specific mapping of the human SUMO proteome reveals co-modification with phosphorylation. Nat. Struct. Mol. Biol. · 2017
… and 30 more in the literature graph

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