InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Protein: CLNS1A

Summary

InnateDB Protein

IDBP-603866.3

Last Modified

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

Gene Symbol

CLNS1A

Protein Name

chloride channel, nucleotide-sensitive, 1A

Synonyms

CLCI; CLNS1B; ICln;

Species

Homo sapiens

Ensembl Protein

ENSP00000433919

InnateDB Gene

IDBG-65661 (CLNS1A)

Protein Structure

UniProt Annotation

Function

Chaperone that regulates the assembly of spliceosomal U1, U2, U4 and U5 small nuclear ribonucleoproteins (snRNPs), the building blocks of the spliceosome. Thereby, plays an important role in the splicing of cellular pre-mRNAs. Most spliceosomal snRNPs contain a common set of Sm proteins SNRPB, SNRPD1, SNRPD2, SNRPD3, SNRPE, SNRPF and SNRPG that assemble in a heptameric protein ring on the Sm site of the small nuclear RNA to form the core snRNP. In the cytosol, the Sm proteins SNRPD1, SNRPD2, SNRPE, SNRPF and SNRPG are trapped in an inactive 6S pICln-Sm complex by the chaperone CLNS1A that controls the assembly of the core snRNP. Dissociation by the SMN complex of CLNS1A from the trapped Sm proteins and their transfer to an SMN-Sm complex triggers the assembly of core snRNPs and their transport to the nucleus. May also indirectly participate in cellular volume control by activation of a swelling-induced chloride conductance pathway. {ECO:0000269PubMed:10330151, ECO:0000269PubMed:11713266, ECO:0000269PubMed:18984161}.

Subcellular Localization

Cytoplasm, cytosol {ECO:0000269PubMed:18984161}. Nucleus {ECO:0000269PubMed:18984161}. Cytoplasm, cytoskeleton {ECO:0000269PubMed:18984161}. Note=A small fraction is also associated with the cytoskeleton.

Disease Associations

Tissue Specificity

Comments

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 94 experimentally validated interaction(s) in this database.

Experimentally validated
Total	94 [view]
Protein-Protein	93 [view]
Protein-DNA	1 [view]
Protein-RNA	0
DNA-DNA	0
RNA-RNA	0
DNA-RNA	0

Gene Ontology

Molecular Function

Accession	GO Term
GO:0005515	protein binding
GO:0044822	poly(A) RNA binding

Biological Process

GO:0000387	spliceosomal snRNP assembly
GO:0006821	chloride transport
GO:0006884	cell volume homeostasis
GO:0010467	gene expression
GO:0016070	RNA metabolic process
GO:0034660	ncRNA metabolic process

Cellular Component

GO:0005634	nucleus
GO:0005737	cytoplasm
GO:0005829	cytosol
GO:0005856	cytoskeleton
GO:0005886	plasma membrane
GO:0034709	methylosome
GO:0034715	pICln-Sm protein complex