ICLN iso2 Involved in both the assembly of spliceosomal snRNPs and the methylation of Sm proteins. 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. Belongs to the pICln (TC 1.A.47) family. Note: This description may include information from UniProtKB.
Protein type: Channel, chloride; RNA splicing
Chromosomal Location of Human Ortholog: 7 E1|7 53.57 cM
Cellular Component:  cytoplasm; cytoskeleton; cytosol; methylosome; nucleoplasm; nucleus; pICln-Sm protein complex; plasma membrane
Molecular Function:  protein heterodimerization activity
Biological Process:  cell volume homeostasis; chloride transport; negative regulation of cell volume; spliceosomal snRNP assembly
Reference #:  Q923F1 (UniProtKB)
Alt. Names/Synonyms: 2610036D06Rik; 2610100O04Rik; chloride channel current inducer; chloride channel regulator; chloride channel, nucleotide sensitive 1A; Chloride channel, nucleotide-sensitive, 1A; Chloride channel, nucleotide-sensitive, 1A, isoform CRA_b; chloride conductance regulatory protein ICln; chloride ion current inducer protein; Clci; Clcni; Clns1a; i(Cln); ICLN; ICLN iso2; methylosome subunit pICln; OTTMUSP00000029289
Gene Symbols: Clns1a
Molecular weight: 26,523 Da
Basal Isoelectric point: 3.98  Predict pI for various phosphorylation states
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ICLN iso2

Protein Structure Not Found.


Cross-references to other databases:  STRING  |  Reactome  |  BioGPS  |  Pfam  |  Phospho.ELM  |  NetworKIN  |  UniProtKB  |  Entrez-Gene  |  Ensembl Gene