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Protein Page:
PQBP1 (human)
p Phosphorylation
ac Acetylation
me Methylation
m1 Mono-methylation
m2 Di-methylation
m3 Tri-methylation
ub Ubiquitination
sm Sumoylation
ne Neddylation
gl O-GlcNAc
ga O-GalNAc
pa Palmitoylation
ad Adenylylation
sn S-Nitrosylation
ca Caspase cleavage
sc Succinylation

Overview
PQBP1 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. May be involved in ATXN1 mutant-induced cell death. The interaction with ATXN1 mutant reduces levels of phosphorylated RNA polymerase II large subunit. Defects in PQBP1 are the cause of Renpenning syndrome 1 (RENS1); also known as Sutherland-Haan X-linked mental retardation syndrome (SHS) or X-linked mental retardation syndromes MRXS3/MRXS8/MRX55. The clinical features are mental retardation, microcephaly, short stature, and small testes. The craniofacies tends to be narrow and tall with upslanting palpebral fissures, abnormal nasal configuration, cupped ears, and short philtrum. The nose may appear long or bulbous, with overhanging columella. Less consistent manifestations include ocular colobomas, cardiac malformations, cleft palate, and anal anomalies. RENS1 is more frequently in males than in females where little or no expression is found. 10 isoforms of the human protein are produced by alternative splicing. Note: This description may include information from UniProtKB.
Protein type: Transcription, coactivator/corepressor
Cellular Component: stress granule; cytoplasm; nucleolus; nucleus
Molecular Function: ribonucleoprotein binding; DNA binding; transcription coactivator activity
Biological Process: regulation of transcription, DNA-dependent; transcription, DNA-dependent; regulation of RNA splicing
Reference #:  O60828 (UniProtKB)
Alt. Names/Synonyms: 38 kDa nuclear protein containing a WW domain; MRX55; MRXS3; MRXS8; NPW38; nuclear protein containing WW domain 38 kD; polyglutamine binding protein 1; Polyglutamine tract-binding protein 1; Polyglutamine-binding protein 1; PQBP-1; PQBP1; RENS1; SHS
Gene Symbols: PQBP1
Molecular weight: 30,472 Da
Basal Isoelectric point: 5.92  Predict pI for various phosphorylation states
Select Structure to View Below

PQBP1

Protein Structure Not Found.


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Modification Sites and Domains Show Modification Legend
Click here to view phosphorylation modifications only

Modification Sites in Parent Protein, Orthologs, and Isoforms Show Modification Legend
 

Show Multiple Sequence Alignment


 SS 

SS: The number of records in which this modification site was determined using site-specific methods. SS methods include amino acid sequencing, site-directed mutagenesis, modification site-specific antibodies, specific MS strategies, etc.


 MS 

MS: The number of records in which this modification site was assigned using ONLY proteomic discovery-mode mass spectrometry.


       human

► Hide Isoforms
 
0 1 - gap
0 1 T9 PLPVALQTrLAKRGI
0 1 R10-m1 LPVALQTrLAKRGIL
0 16 Y33-p EEIIAEDyDDDPVDY
0 1 S94-p KSAKKLRsSNADAEE
0 8 Y146-p DRDRERGyDKVDRER
0 13 Y209-p DPMDPSSysDAPRGT
0 1 S210-p PMDPSSysDAPRGTW
0 1 T216 ysDAPRGTWSTGLPK
0 8 S247-p FQQRPYPsPGAVLRA
  PQBP1 iso3  
- gap
T9 PLPVALQTRLAKRGI
R10 LPVALQTRLAKRGIL
Y33-p EEIIAEDyDDDPVDY
S94 KSAKKLRSSNADAEE
Y146 DRDRERGYDKVDRER
- gap
- gap
- gap
- gap
  PQBP1 iso11  
S3-p _____MLsAMPLPVA
T13-p PLPVALQtRLAKRGI
R14 LPVALQtRLAKRGIL
Y37 EEIIAEDYDDDPVDY
S98 KSAKKLRSSNADAEE
- gap
- gap
- gap
- gap
- gap
  mouse

 
- gap
T9 PLPVALQTRLAKRGI
R10 LPVALQTRLAKRGIL
Y33 EEIIAEDYDDDPVDY
N94 KSAKKVRNNNADAED
Y146 DRERERNYDKVDRER
Y207 DPMDPSSYSDAPRGt
S208 PMDPSSYSDAPRGtW
T214-p YSDAPRGtWSTGLPK
S245 FQQRPYPSPGAVLRA
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