InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Protein: SNRNP200

Summary

InnateDB Protein

IDBP-61604.6

Last Modified

2014-10-13 [Report errors or provide feedback]

Gene Symbol

SNRNP200

Protein Name

small nuclear ribonucleoprotein 200kDa (U5)

Synonyms

ASCC3L1; BRR2; HELIC2; RP33; U5-200KD;

Species

Homo sapiens

Ensembl Protein

ENSP00000317123

InnateDB Gene

IDBG-61602 (SNRNP200)

Protein Structure

UniProt Annotation

Function

RNA helicase that plays an essential role in pre-mRNA splicing as component of the U5 snRNP and U4/U6-U5 tri-snRNP complexes. Involved in spliceosome assembly, activation and disassembly. Mediates changes in the dynamic network of RNA-RNA interactions in the spliceosome. Catalyzes the ATP-dependent unwinding of U4/U6 RNA duplices, an essential step in the assembly of a catalytically active spliceosome. {ECO:0000269PubMed:16723661, ECO:0000269PubMed:23045696, ECO:0000269PubMed:8670905, ECO:0000269PubMed:9539711}.

Subcellular Localization

Nucleus {ECO:0000269PubMed:9539711}.

Disease Associations

Retinitis pigmentosa 33 (RP33) [MIM:610359]: A retinal dystrophy belonging to the group of pigmentary retinopathies. Retinitis pigmentosa is characterized by retinal pigment deposits visible on fundus examination and primary loss of rod photoreceptor cells followed by secondary loss of cone photoreceptors. Patients typically have night vision blindness and loss of midperipheral visual field. As their condition progresses, they lose their far peripheral visual field and eventually central vision as well. {ECO:0000269PubMed:16723661, ECO:0000269PubMed:19710410, ECO:0000269PubMed:19878916, ECO:0000269PubMed:21618346}. Note=The disease is caused by mutations affecting the gene represented in this entry.

Tissue Specificity

Widely expressed. {ECO:0000269PubMed:19878916}.

Comments

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 157 experimentally validated interaction(s) in this database.

Experimentally validated
Total	157 [view]
Protein-Protein	156 [view]
Protein-DNA	0
Protein-RNA	0
DNA-DNA	1 [view]
RNA-RNA	0
DNA-RNA	0

Gene Ontology

Molecular Function

Accession	GO Term
GO:0003676	nucleic acid binding
GO:0003677	DNA binding
GO:0004004	ATP-dependent RNA helicase activity
GO:0005515	protein binding
GO:0005524	ATP binding
GO:0008026	ATP-dependent helicase activity
GO:0016787	hydrolase activity
GO:0042802	identical protein binding
GO:0044822	poly(A) RNA binding

Biological Process

GO:0000354	cis assembly of pre-catalytic spliceosome
GO:0000398	mRNA splicing, via spliceosome
GO:0001649	osteoblast differentiation
GO:0006200	ATP catabolic process
GO:0008380	RNA splicing
GO:0010467	gene expression

Cellular Component

GO:0005634	nucleus
GO:0005654	nucleoplasm
GO:0005681	spliceosomal complex
GO:0005682	U5 snRNP
GO:0016020	membrane
GO:0071013	catalytic step 2 spliceosome

Protein Structure and Domains

PDB ID

InterPro

IPR001650 Helicase, C-terminal
IPR004179 Sec63 domain
IPR006935 Helicase/UvrB domain
IPR011545 DEAD/DEAH box helicase domain
IPR014001 Helicase, superfamily 1/2, ATP-binding domain
IPR027417 P-loop containing nucleoside triphosphate hydrolase

PFAM

PF00271
PF02889
PF04851
PF00270

PRINTS

PIRSF

SMART

SM00490
SM00611
SM00973
SM00487

TIGRFAMs

Post-translational Modifications

Modification

Cross-References

SwissProt

O75643