UniProt:O60828 PQBP1

chain
  • initiator methionine:1
  • chain:2-265
checksum 98C3BEF18CFF0297
comment
  • FUNCTION Intrinsically disordered protein that acts as a scaffold, and which is involved in different processes, such as pre-mRNA splicing, transcription regulation, innate immunity and neuron development (PubMed:10198427, PubMed:10332029, PubMed:12062018, PubMed:20410308, PubMed:23512658). Interacts with splicing-related factors via the intrinsically disordered region and regulates alternative splicing of target pre-mRNA species (PubMed:10332029, PubMed:12062018, PubMed:20410308, PubMed:23512658). May suppress the ability of POU3F2 to transactivate the DRD1 gene in a POU3F2 dependent manner. Can activate transcription directly or via association with the transcription machinery (PubMed:10198427). May be involved in ATXN1 mutant-induced cell death (PubMed:12062018). The interaction with ATXN1 mutant reduces levels of phosphorylated RNA polymerase II large subunit (PubMed:12062018). Involved in the assembly of cytoplasmic stress granule, possibly by participating in the transport of neuronal RNA granules (PubMed:21933836). Also acts as an innate immune sensor of infection by retroviruses, such as HIV, by detecting the presence of reverse-transcribed DNA in the cytosol (PubMed:26046437). Directly binds retroviral reverse-transcribed DNA in the cytosol and interacts with CGAS, leading to activate the cGAS-STING signaling pathway, triggering type-I interferon production (PubMed:26046437).SUBUNIT Interacts with POU3F2/Brn-2, ATXN1, TXNL4A, HTT and AR (PubMed:10332029, PubMed:10873650, PubMed:19303059, PubMed:24781215). Interaction with ATXN1 correlates positively with the length of the polyglutamine tract (PubMed:12062018). Interacts with RNA polymerase II large subunit in a phosphorylation-dependent manner (PubMed:12062018). Forms a ternary complex with ATXN1 mutant and phosphorylated RNA polymerase II (PubMed:12062018). Interacts (via C-terminus) with TXNL4A and CD2BP2 (PubMed:10873650, PubMed:19303059, PubMed:24781215). Interacts (via WW domain) with ATN1 and SF3B1, and may interact with additional splice factors (PubMed:20410308, PubMed:23512658). Interacts (via WW domain) with WBP11; Leading to reduce interaction between PQBP1 and TXNL4A (PubMed:20410308, PubMed:23512658, PubMed:27314904). Interacts with CAPRIN1 (PubMed:21933836). Interacts with DDX1 (PubMed:21933836). Interacts with SFPQ (PubMed:21933836). Interacts with KHSRP (PubMed:21933836).INTERACTION Colocalizes with SRSF2 in nuclear speckles (By similarity). Colocalized with POU3F2 (PubMed:10332029). Colocalized with ATXN1 in nuclear inclusion bodies (PubMed:12062018). Localizes to cytoplasmic stress granules (PubMed:21933836).ALTERNATIVE PRODUCTS Widely expressed with high level in heart, skeletal muscle, pancreas, spleen, thymus, prostate, ovary, small intestine and peripheral blood leukocytes.DOMAIN The WW domain may play a role as a transcriptional activator directly or via association with the transcription machinery. The WW domain mediates interaction with WBP11, ATN1, SF3B1 and the C-terminal domain of the RNA polymerase II large subunit.DOMAIN Except for the WW domain, the protein is intrinsically disordered.DISEASE The disease is caused by variants affecting the gene represented in this entry.
created [InstanceEdit:217385] Schmidt, EE, 2008-03-27 06:23:53
crossReference
databaseName UniProt
dbId 243721
description
  • recommendedName: fullName evidence="23 24"Polyglutamine-binding protein 1 shortName evidence="23 24"PQBP-1 alternativeName: fullName evidence="22"38 kDa nuclear protein containing a WW domain shortName evidence="22"Npw38 alternativeName: fullName evidence="23"Polyglutamine tract-binding protein 1
displayName UniProt:O60828 PQBP1
geneName
  • PQBP1
  • NPW38
  • JM26
identifier O60828
isSequenceChanged false
keyword
  • 3D-structure
  • Alternative splicing
  • Direct protein sequencing
  • Immunity
  • Innate immunity
  • Intellectual disability
  • mRNA processing
  • mRNA splicing
  • Nucleus
  • Phosphoprotein
  • Proteomics identification
  • Reference proteome
  • Repeat
  • Transcription
  • Transcription regulation
modified [InstanceEdit:9963647] Weiser, Joel, 2025-08-15
moleculeType Protein
name
  • PQBP1
otherIdentifier
  • 10084
  • 11730026_x_at
  • 11753989_x_at
  • 11756030_a_at
  • 11761355_a_at
  • 11761356_x_at
  • 17103509
  • 207769_PM_s_at
  • 207769_s_at
  • 210499_3p_s_at
  • 210499_PM_s_at
  • 210499_s_at
  • 214527_PM_s_at
  • 214527_s_at
  • 34325_at
  • 3976931
  • 3976932
  • 3976933
  • 3976934
  • 3976935
  • 3976936
  • 3976937
  • 3976938
  • 3976939
  • 3976940
  • 3976941
  • 3976942
  • 3976943
  • 3976944
  • 3976945
  • 3976946
  • 3976947
  • 3976948
  • 3976949
  • 8167412
  • A_14_P111129
  • A_14_P139896
  • A_23_P62139
  • A_33_P3224371
  • A_33_P3325933
  • A_33_P3325935
  • GE55166
  • GO:0000380
  • GO:0002218
  • GO:0002230
  • GO:0002376
  • GO:0003677
  • GO:0003690
  • GO:0003713
  • GO:0005515
  • GO:0005634
  • GO:0005654
  • GO:0005737
  • GO:0005815
  • GO:0005829
  • GO:0005856
  • GO:0005929
  • GO:0006355
  • GO:0006397
  • GO:0008380
  • GO:0010494
  • GO:0015630
  • GO:0016071
  • GO:0016604
  • GO:0016607
  • GO:0031175
  • GO:0032481
  • GO:0036064
  • GO:0043021
  • GO:0043226
  • GO:0043484
  • GO:0045087
  • GO:0045893
  • GO:0048814
  • GO:0051607
  • GO:0071360
  • GO:0071598
  • GO:0098542
  • GO:0120025
  • HMNXSV003037667
  • Hs.30570.4.S1_3p_a_at
  • Hs.30570.4.S1_3p_x_at
  • ILMN_1692429
  • ILMN_1711596
  • ILMN_2325234
  • PH_hs_0002613
  • TC0X000263.hg
  • g10801589_3p_a_at
  • g10801589_3p_at
  • g10801589_3p_x_at
physicalEntity
referenceDatabase [ReferenceDatabase:2] UniProt
referenceGene
referenceTranscript
schemaClass ReferenceGeneProduct
secondaryIdentifier
  • PQBP1_HUMAN
  • C9JQA1
  • Q4VY25
  • Q4VY26
  • Q4VY27
  • Q4VY29
  • Q4VY30
  • Q4VY34
  • Q4VY35
  • Q4VY36
  • Q4VY37
  • Q4VY38
  • Q9GZP2
  • Q9GZU4
  • Q9GZZ4
sequenceLength 265
species [Species:48887] Homo sapiens
stId uniprot:O60828
url http://purl.uniprot.org/uniprot/O60828
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