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PQBP1, Antibody

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產(chǎn)品名稱(chēng): PQBP1, Antibody
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PQBP1, Antibody


PQBP1, Antibody  的詳細(xì)介紹
Product Name

PQBP1, Antibody

Full Product Name

PQBP1 Antibody

Product Gene Name

anti-PQBP1 antibody

[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
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OMIM
300463
3D Structure
ModBase 3D Structure for O60828
Other Notes
Small volumes of anti-PQBP1 antibody 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.
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NCBI/Uniprot data below describe general gene information for PQBP1. It may not necessarily be applicable to this product.
NCBI GI #
74027247
NCBI GeneID
10084
NCBI Accession #
NP_001027553.1 [Other Products]
NCBI GenBank Nucleotide #
NM_001032381.1 [Other Products]
UniProt Primary Accession #
O60828 [Other Products]
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
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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]
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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]
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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. The WW domain belonging to polyglutamine tract-binding protein 1 (PQBP1) is of particular interest due to its direct involvement in several X chromosome-linked intellectual disabilities, including Golabi-Ito-Hall (GIH) syndrome, where a single point mutation (Y65C) correlates with the development of the disease. The mutant cannot bind to its natural ligand WBP11, which regulates mRNA processing
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Precautions
All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
Disclaimer
While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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