UniProt:P83369 LSM11

chain
  • chain:1-360
checksum 3C97710C28C0DCE1
comment
  • FUNCTION Component of the U7 snRNP complex that is involved in the histone 3'-end pre-mRNA processing (PubMed:11574479, PubMed:16914750, PubMed:33230297). Increases U7 snRNA levels but not histone 3'-end pre-mRNA processing activity, when overexpressed (PubMed:11574479, PubMed:16914750). Required for cell cycle progression from G1 to S phases (By similarity). Binds specifically to the Sm-binding site of U7 snRNA (PubMed:11574479, PubMed:16914750).SUBUNIT Component of the heptameric ring U7 snRNP complex, or U7 Sm protein core complex, at least composed of LSM10, LSM11, SNRPB, SNRPD3, SNRPE, SNRPF, SNRPG and U7 snRNA (PubMed:11574479, PubMed:12975319). Formation of the U7 snRNP is an ATP-dependent process mediated by a specialized SMN complex containing at least the Sm protein core complex and additionally, the U7-specific LSM10 and LSM11 proteins (PubMed:11574479). Identified in a histone pre-mRNA complex, at least composed of ERI1, LSM11, SLBP, SNRPB, SYNCRIP and YBX1 (By similarity). Interacts (via the Sm domains) with CLNS1A (By similarity). Interacts with SMN and ZNF473 (PubMed:12975319, PubMed:16714279). Interacts with PRMT5 and WDR77 (By similarity).INTERACTION The C-terminal SM 1 domain is both necessary for the binding to the Sm-binding site of U7 snRNA and U7 snRNP assembly (By similarity). The N-terminal domain is essential for histone pre-mRNA cleavage (By similarity). Amino acids 63-82 are sufficient to interact with ZNF473 (By similarity).DISEASE The disease is caused by variants affecting the gene represented in this entry. Impaired histone 3'-end pre-mRNA processing caused by disease variants affects chromatin structure, relieving CGAS inhibition by nucleosomes (PubMed:33230297). This activates the cGAS-STING pathway, triggering type-I interferon production and autoinflammation (PubMed:33230297).SIMILARITY Belongs to the snRNP Sm proteins family.
crossReference
databaseName UniProt
dbId 94627
description
  • recommendedName: fullName evidence="11"U7 snRNA-associated Sm-like protein LSm11
displayName UniProt:P83369 LSM11
geneName
  • LSM11
identifier P83369
isSequenceChanged false
keyword
  • 3D-structure
  • Aicardi-Goutieres syndrome
  • Direct protein sequencing
  • Disease variant
  • Isopeptide bond
  • Methylation
  • mRNA processing
  • Nucleus
  • Phosphoprotein
  • Proteomics identification
  • Reference proteome
  • Repeat
  • Ribonucleoprotein
  • RNA-binding
  • Ubl conjugation
modified [InstanceEdit:9926675] Weiser, Joel, 2024-11-03
moleculeType Protein
name
  • LSM11
otherIdentifier
  • 11734107_a_at
  • 11734108_a_at
  • 11734109_at
  • 134353
  • 16991617
  • 226819_PM_at
  • 226819_at
  • 226826_PM_at
  • 226826_at
  • 241599_PM_at
  • 241599_at
  • 242112_PM_at
  • 242112_at
  • 2837500
  • 2837501
  • 2837502
  • 2837507
  • 2837508
  • 2837509
  • 2837510
  • 2837511
  • 2837513
  • 43457_at
  • 77136_at
  • 8109585
  • 82847_at
  • A_14_P130029
  • A_23_P368195
  • A_32_P196520
  • A_32_P46526
  • GE55325
  • GE584587
  • GO:0003723
  • GO:0005515
  • GO:0005634
  • GO:0005654
  • GO:0005683
  • GO:0005697
  • GO:0006397
  • GO:0006398
  • GO:0016071
  • GO:0016604
  • GO:0071204
  • GO:0071209
  • GO:1900087
  • GO:1902275
  • GO:1990904
  • HMNXSV003043361
  • Hs.170158.0.A1_3p_at
  • Hs.187117.0.S1_3p_at
  • Hs.23648.0.S1_3p_at
  • Hs.23648.0.S2_3p_at
  • ILMN_1702698
  • PH_hs_0022043
  • PH_hs_0039864
  • TC05000873.hg
  • p26323
physicalEntity
referenceDatabase [ReferenceDatabase:2] UniProt
referenceGene
referenceTranscript
schemaClass ReferenceGeneProduct
secondaryIdentifier
  • LSM11_HUMAN
  • A0AVQ1
  • Q7Z7P0
  • Q8N975
sequenceLength 360
species [Species:48887] Homo sapiens
stId uniprot:P83369
url http://purl.uniprot.org/uniprot/P83369
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