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MGP000591

UniProt Annotations

Entry Information
Gene Namechloride channel, nucleotide-sensitive, 1A
Protein EntryICLN_HUMAN
UniProt IDP54105
SpeciesHuman
Comments
Comment typeDescription
FunctionChaperone 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:0000269|PubMed:10330151, ECO:0000269|PubMed:11713266, ECO:0000269|PubMed:18984161}.
InteractionO14744:PRMT5; NbExp=3; IntAct=EBI-724693, EBI-351098; P14678:SNRPB; NbExp=3; IntAct=EBI-724693, EBI-372458; P62314:SNRPD1; NbExp=6; IntAct=EBI-724693, EBI-372177; P62316:SNRPD2; NbExp=5; IntAct=EBI-724693, EBI-297993; P62318:SNRPD3; NbExp=6; IntAct=EBI-724693, EBI-372789; P62306:SNRPF; NbExp=4; IntAct=EBI-724693, EBI-356900;
SimilarityBelongs to the pICln (TC 1.A.47) family. {ECO:0000305}.
Subcellular LocationCytoplasm, cytosol {ECO:0000269|PubMed:18984161}. Nucleus {ECO:0000269|PubMed:18984161}. Cytoplasm, cytoskeleton {ECO:0000269|PubMed:18984161}. Note=A small fraction is also associated with the cytoskeleton.
SubunitComponent of the methylosome, a 20S complex containing at least CLNS1A/pICln, PRMT5/SKB1 and WDR77/MEP50; may mediate SNRPD1 and SNRPD3 methylation. Forms a 6S pICln-Sm complex composed of CLNS1A/pICln, SNRPD1, SNRPD2, SNRPE, SNRPF and SNRPG; ring-like structure where CLNS1A/pICln mimics additional Sm proteins and which is unable to assemble into the core snRNP. Interacts with LSM10 and LSM11. {ECO:0000269|PubMed:11747828, ECO:0000269|PubMed:16087681, ECO:0000269|PubMed:18984161}.
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