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Protein

Methylosome subunit pICln

Gene

CLNS1A

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

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.3 Publications

GO - Molecular functioni

  • protein heterodimerization activity Source: MGI
  • RNA binding Source: UniProtKB

GO - Biological processi

  • cell volume homeostasis Source: InterPro
  • chloride transport Source: InterPro
  • spliceosomal snRNP assembly Source: UniProtKB

Enzyme and pathway databases

ReactomeiR-HSA-191859. snRNP Assembly.

Protein family/group databases

TCDBi1.A.47.1.1. the nucleotide-sensitive anion-selective channel, icln (icln) family.

Names & Taxonomyi

Protein namesi
Recommended name:
Methylosome subunit pICln
Alternative name(s):
Chloride channel, nucleotide sensitive 1A
Chloride conductance regulatory protein ICln
Short name:
I(Cln)
Chloride ion current inducer protein
Short name:
ClCI
Reticulocyte pICln
Gene namesi
Name:CLNS1A
Synonyms:CLCI, ICLN
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 11

Organism-specific databases

EuPathDBiHostDB:ENSG00000074201.8.
HGNCiHGNC:2080. CLNS1A.

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Cytoplasm, Cytoskeleton, Nucleus

Pathology & Biotechi

Organism-specific databases

DisGeNETi1207.
OpenTargetsiENSG00000074201.
PharmGKBiPA26607.

Polymorphism and mutation databases

BioMutaiCLNS1A.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Initiator methionineiRemovedCombined sources1 Publication
ChainiPRO_00001851552 – 237Methylosome subunit pIClnAdd BLAST236

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei2N-acetylserineCombined sources1 Publication1
Modified residuei102PhosphoserineCombined sources2 Publications1
Modified residuei144PhosphoserineCombined sources1
Modified residuei193PhosphoserineCombined sources1
Modified residuei195PhosphoserineCombined sources1
Modified residuei198PhosphoserineCombined sources1
Modified residuei210PhosphoserineCombined sources1
Modified residuei223PhosphothreonineCombined sources1

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

EPDiP54105.
MaxQBiP54105.
PaxDbiP54105.
PeptideAtlasiP54105.
PRIDEiP54105.

PTM databases

iPTMnetiP54105.
PhosphoSitePlusiP54105.

Expressioni

Gene expression databases

BgeeiENSG00000074201.
CleanExiHS_CLNS1A.
ExpressionAtlasiP54105. baseline and differential.
GenevisibleiP54105. HS.

Organism-specific databases

HPAiCAB017840.
HPA031707.
HPA031708.
HPA032045.

Interactioni

Subunit structurei

Component 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 LSM11.3 Publications

Binary interactionsi

Show more details

GO - Molecular functioni

  • protein heterodimerization activity Source: MGI

Protein-protein interaction databases

BioGridi107617. 98 interactors.
CORUMiP54105.
DIPiDIP-44185N.
IntActiP54105. 33 interactors.
MINTiMINT-5002627.
STRINGi9606.ENSP00000433919.

Structurei

3D structure databases

ProteinModelPortaliP54105.
SMRiP54105.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Phylogenomic databases

eggNOGiKOG3238. Eukaryota.
ENOG4111R94. LUCA.
GeneTreeiENSGT00390000010063.
HOGENOMiHOG000006913.
HOVERGENiHBG003108.
InParanoidiP54105.
KOiK05019.
OMAiCIYFMLD.
OrthoDBiEOG091G0XFJ.
PhylomeDBiP54105.
TreeFamiTF315155.

Family and domain databases

InterProiView protein in InterPro
IPR003521. ICln.
PRINTSiPR01348. ICLNCHANNEL.

Sequencei

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

P54105-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MSFLKSFPPP GPAEGLLRQQ PDTEAVLNGK GLGTGTLYIA ESRLSWLDGS
60 70 80 90 100
GLGFSLEYPT ISLHALSRDR SDCLGEHLYV MVNAKFEEES KEPVADEEEE
110 120 130 140 150
DSDDDVEPIT EFRFVPSDKS ALEAMFTAMC ECQALHPDPE DEDSDDYDGE
160 170 180 190 200
EYDVEAHEQG QGDIPTFYTY EEGLSHLTAE GQATLERLEG MLSQSVSSQY
210 220 230
NMAGVRTEDS IRDYEDGMEV DTTPTVAGQF EDADVDH
Length:237
Mass (Da):26,215
Last modified:October 1, 1996 - v1
Checksum:iAE9C09884A6FF158
GO

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_01573620Q → H1 Publication1
Natural variantiVAR_015737218M → T1 Publication1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
X91788 mRNA. Translation: CAA62902.1.
U17899 mRNA. Translation: AAC50111.1.
U53454 mRNA. Translation: AAB03316.1.
AF005422 mRNA. Translation: AAB61444.1.
AF026003 mRNA. Translation: AAB88806.1.
AF232708 Genomic DNA. Translation: AAF76861.1.
AF232224 Genomic DNA. Translation: AAF76858.1.
AF232225 Genomic DNA. Translation: AAF76859.1.
AK315259 mRNA. Translation: BAG37676.1.
BT019907 mRNA. Translation: AAV38710.1.
BC119634 mRNA. Translation: AAI19635.1.
BC119635 mRNA. Translation: AAI19636.1.
CCDSiCCDS8252.1.
PIRiJC4135.
RefSeqiNP_001284.1. NM_001293.2.
NP_001298128.1. NM_001311199.1.
NP_001298129.1. NM_001311200.1.
NP_001298130.1. NM_001311201.1.
NP_001298131.1. NM_001311202.1.
UniGeneiHs.430733.

Genome annotation databases

EnsembliENST00000525428; ENSP00000433919; ENSG00000074201.
ENST00000528364; ENSP00000434311; ENSG00000074201.
GeneIDi1207.
KEGGihsa:1207.
UCSCiuc001oyk.4. human.

Keywords - Coding sequence diversityi

Polymorphism

Similar proteinsi

Entry informationi

Entry nameiICLN_HUMAN
AccessioniPrimary (citable) accession number: P54105
Secondary accession number(s): B2RCS9
, Q0VDK6, Q9NRD2, Q9NRD3
Entry historyiIntegrated into UniProtKB/Swiss-Prot: October 1, 1996
Last sequence update: October 1, 1996
Last modified: October 25, 2017
This is version 163 of the entry and version 1 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. Human chromosome 11
    Human chromosome 11: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. SIMILARITY comments
    Index of protein domains and families