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Mouse Anti-PQBP1 Recombinant Antibody (3H7) (CBMAB-P2678-YC)

Provided herein is a Mouse monoclonal antibody against Human Polyglutamine Binding Protein 1. The antibody can be used for immunoassay techniques, such as ELISA, IF, WB.
See all PQBP1 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
3H7
Antibody Isotype
IgG2a, κ
Application
ELISA, IF, WB

Basic Information

Immunogen
PQBP1 (AAH12358, 1-265 aa) full length recombinant protein with GST tag. Immunogen sequence: MPLPVALQTR LAKRGILKHL EPEPEEEIIA EDYDDDPVDY EATRLEGLPP SWYKVFDPSC GLPYYWNADT DLVSWLSPHD PNSVVTKSAK KLRSSNADAE EKLDRSHDKS DRGHDKSDRS HEKLDRGHDK SDRGHDKSDR DRERGYDKVD RERERDRERD RDRGYDKADR EEGKERRHHR REELAPYPKS KKAVSRKDEE LDPMDPSSYS DAPRGTWSTG LPKRNEAKTG ADTTAAGPLF QQRPYPSPGA VLRANAEASR TKQQD
Specificity
Human
Antibody Isotype
IgG2a, κ
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Storage
Store at 4°C short term (1-2 weeks). Aliquot and store at-20°C long term. Avoid repeated freeze/thaw cycles.
Epitope
aa 1-265

Target

Full Name
polyglutamine binding protein 1
Introduction
PQBP1 (Polyglutamine Binding Protein 1) is a protein coding gene. Diseases associated with PQBP1 include Renpenning Syndrome 1 and X-Linked Intellectual Disability, Golabi-Ito-Hall Type. Among its related pathways are mRNA Splicing-Major Pathway and Gene Expression. Gene Ontology annotations related to this gene include transcription coactivator activity and ribonucleoprotein complex binding.
Entrez Gene ID
10084
UniProt ID
O60828
Alternative Names
MRX2; MRX55; MRXS3; MRXS8; NPW38; RENS1; SHS
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: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 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).
Biological Process
Activation of innate immune responseManual Assertion Based On ExperimentIDA:UniProtKB
Alternative mRNA splicing, via spliceosomeManual Assertion Based On ExperimentIMP:UniProtKB
Cellular response to exogenous dsRNAManual Assertion Based On ExperimentIDA:UniProtKB
Defense response to virusManual Assertion Based On ExperimentIDA:UniProtKB
Innate immune responseIEA:UniProtKB-KW
Neuron projection developmentISS:UniProtKB
Positive regulation of defense response to virus by hostManual Assertion Based On ExperimentIDA:UniProtKB
Positive regulation of type I interferon productionManual Assertion Based On ExperimentIDA:UniProtKB
Regulation of dendrite morphogenesisIEA:Ensembl
Regulation of RNA splicingManual Assertion Based On ExperimentIMP:MGI
Regulation of transcription, DNA-templatedManual Assertion Based On ExperimentTAS:ProtInc
Cellular Location
Nucleus
Nucleus speckle
Cytoplasmic granule
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).
Involvement in disease
Renpenning syndrome 1 (RENS1):
An X-linked syndrome characterized by intellectual disability, 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.
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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