| 靶标 | CLCI, CLNS1A, Chloride channel, nucleotide sensitive 1A, Chloride conductance regulatory protein ICln, Chloride ion current inducer protein, ClCI, I(Cln), ICLN, Methylosome subunit pICln, Reticulocyte pICln |
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| 背景 | Methylosome subunit pICln (CLNS1A) is a ~26 kDa protein. 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, and 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 (Sm core). 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. 1. Pu, WT. et al. (1999) Molecular and cellular biology 19, 4113-20. PMID: 103301512. Friesen, WJ. et al. (2001) Molecular and cellular biology 21, 8289-300. PMID: 117132664. Guderian, G. et al. (2011) The Journal of biological chemistry 286, 1976-86. PMID: 21081503 |
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