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Anti-PQBP1 Antibody (CAB4369)

SKU:
CAB4369
Product Type:
Antibody
Antibody Type:
Polyclonal Antibody
Reactivity:
Human
Mouse
Rat
Host Species:
Rabbit
Isotype:
IgG
Synonyms:
PQBP1
MRX2
MRX55
MRXS3
MRXS8
NPW38
RENS1
SHS
Research Area:
Immunology
  • Immunology Antibodies 2 Anti-PQBP1 Antibody CAB4369
  • Immunology Antibodies 2 Anti-PQBP1 Antibody CAB4369
€139 - €419
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Description

Product Name:PQBP1 Rabbit pAb
Product Code:CAB4369
Size:20uL, 50uL, 100uL
Synonyms:PQBP1, MRX2, MRX55, MRXS3, MRXS8, NPW38, RENS1, SHS
Applications:WB
Reactivity:Human, Mouse, Rat
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 1-265 of human PQBP1 (NP_005701.1).
Applications:WB
Recommended Dilutions:WB 1:200 - 1:2000
Reactivity:Human, Mouse, Rat
Positive Samples:22Rv1, SW480, HL-60, Mouse liver, Mouse brain, Rat brain, Rat ovary, Rat liver, SKOV3
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 1-265 of human PQBP1 (NP_005701.1).
Purification Method:Affinity purification
Storage:Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:MPLP VALQ TRLA KRGI LKHL EPEP EEEI IAED YDDD PVDY EATR LEGL PPSW YKVF DPSC GLPY YWNA DTDL VSWL SPHD PNSV VTKS AKKL RSSN ADAE EKLD RSHD KSDR GHDK SDRS HEKL DRGH DKSD RGHD KSDR DRER GYDK VDRE RERD RERD RDRG YDKA DREE GKER RHHR REEL APYP KSKK AVSR KDEE LDPM DPSS YSDA PRGT WSTG LPKR NEAK TGAD TTAA GPLF QQRP YPSP GAVL RANA EASR TKQQ D
Gene ID:10084
Uniprot:O60828
Cellular Location:Nucleus, Nucleus speckle
Calculated MW:6kDa/7kDa/8kDa/14kDa/17kDa/18kDa/26kDa/30kDa
Observed MW:30kDa
UniProt Protein Function: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.
UniProt Protein Details:

Protein type:Transcription, coactivator/corepressor

Chromosomal Location of Human Ortholog: Xp11.23

Cellular Component: nucleoplasm; stress granule; cytoplasm; nuclear speck; nucleus

Molecular Function:ribonucleoprotein binding; DNA binding; transcription coactivator activity

Biological Process: alternative nuclear mRNA splicing, via spliceosome; transcription, DNA-dependent; regulation of transcription, DNA-dependent; regulation of RNA splicing; regulation of dendrite morphogenesis; neurite development

Disease: Renpenning Syndrome 1

NCBI Summary:This gene encodes a nuclear polyglutamine-binding protein that is involved with transcription activation. The encoded protein contains a WW domain. Mutations in this gene have been found in patients with Renpenning syndrome 1 and other syndromes with X-linked mental retardation. Multiple alternatively spliced transcript variants that encode different protein isoforms have been described for this gene.[provided by RefSeq, Nov 2009]
UniProt Code:O60828
NCBI GenInfo Identifier:74735456
NCBI Gene ID:10084
NCBI Accession:O60828.1
UniProt Secondary Accession:O60828,Q4VY25, Q4VY26, Q4VY27, Q4VY29, Q4VY30, Q4VY34 Q4VY35, Q4VY36, Q4VY37, Q4VY38, Q9GZP2,
UniProt Related Accession:O60828
Molecular Weight:265
NCBI Full Name:Polyglutamine-binding protein 1
NCBI Synonym Full Names:polyglutamine binding protein 1
NCBI Official Symbol:PQBP1  
NCBI Official Synonym Symbols:SHS; MRX2; MRX55; MRXS3; MRXS8; NPW38; RENS1  
NCBI Protein Information:polyglutamine-binding protein 1; polyglutamine tract-binding protein 1; 38 kDa nuclear protein containing a WW domain
UniProt Protein Name:Polyglutamine-binding protein 1
UniProt Synonym Protein Names:38 kDa nuclear protein containing a WW domain; Npw38; Polyglutamine tract-binding protein 1
Protein Family:Polyglutamine-binding protein
UniProt Gene Name:PQBP1  
UniProt Entry Name:PQBP1_HUMAN

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