Product Name
Polyglutamine-binding protein 1 (PQBP1), Recombinant Protein
Full Product Name
Recombinant Human Polyglutamine-binding protein 1 (PQBP1)
Product Gene Name
PQBP1 recombinant protein
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Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Sequence Positions
2-265aa, Full Length of Mature Protein
Sequence
PLPVALQTRL AKRGILKHLE PEPEEEIIAE DYDDDPVDYE ATRLEGLPPS WYKVFDPSCG LPYYWNADTD LVSWLSPHDP NSVVTKSAKK LRSSNADAEE KLDRSHDKSD RGHDKSDRSH EKLDRGHDKS DRGHDKSDRD RERGYDKVDR ERERDRERDR DRGYDKADRE EGKERRHHRR EELAPYPKSK KAVSRKDEEL DPMDPSSYSD APRGTWSTGL PKRNEAKTGA DTTAAGPLFQ QRPYPSPGAV LRANAEASRT KQQD
3D Structure
ModBase 3D Structure for O60828
Host
E Coli or Yeast or Baculovirus or Mammalian Cell
Purity/Purification
>85% (SDS-PAGE) (lot specific)
Form/Format
Liquid containing glycerol
Tag Information
This protein contains an N-terminal tag and may also contain a C-terminal tag. Tag types are determined by various factors including tag-protein stability, please inquire for tag information.
Sterility
Sterile filter available upon request.
Endotoxin
Low endotoxin available upon request.
Storage Buffer
Tris-based buffer, 50% glycerol
Preparation and Storage
Store at -20 degrees C. For long-term storage, store at -20 degrees C or -80 degrees C. Store working aliquots at 4 degrees C for up to one week. Repeated freezing and thawing is not recommended.
ISO Certification
Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.
Other Notes
Small volumes of PQBP1 recombinant protein vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
NCBI/Uniprot data below describe general gene information for PQBP1. It may not necessarily be applicable to this product.
NCBI Accession #
NP_001027553.1
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NCBI GenBank Nucleotide #
NM_001032381.1
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UniProt Primary Accession #
O60828
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UniProt Secondary Accession #
Q4VY25; Q4VY26; Q4VY27; Q4VY29; Q4VY30; Q4VY34; Q4VY35; Q4VY36; Q4VY37; Q4VY38; C9JQA1[Other Products]
UniProt Related Accession #
O60828[Other Products]
Molecular Weight
6,981 Da
NCBI Official Full Name
polyglutamine-binding protein 1 isoform 1
NCBI Official Synonym Full Names
polyglutamine binding protein 1
NCBI Official Symbol
PQBP1??[Similar Products]
NCBI Official Synonym Symbols
SHS; MRX2; MRX55; MRXS3; MRXS8; NPW38; RENS1
??[Similar Products]
NCBI Protein Information
polyglutamine-binding protein 1
UniProt Protein Name
Polyglutamine-binding protein 1
UniProt Synonym Protein Names
38 kDa nuclear protein containing a WW domain
Protein Family
Polyglutamine-binding protein
UniProt Gene Name
PQBP1??[Similar Products]
UniProt Synonym Gene Names
Npw38??[Similar Products]
NCBI Summary for PQBP1
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 cognitive disability. Multiple alternatively spliced transcript variants that encode different protein isoforms have been described for this gene.[provided by RefSeq, Nov 2009]
UniProt Comments for PQBP1
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:23512658, PubMed:20410308). 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 to 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).
Research Articles on PQBP1
1. Our data strongly support a gain-of-function pathogenic mechanism of PQBP1 c.459_462delAGAG and c.463_464dupAG mutations, and suggest that therapeutic strategies to restore FMRP function may be beneficial for those patients
Precautions
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Disclaimer
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