Polyglutamine binding protein 1; MRX55; MRXS3; MRXS8; NPW38; RENS1; SHS

For Research Use Only. Not for use in diagnostic procedures.

E Coli

> 95% by SDS-PAGE

Liquid. In 20mM Tris-HCl Buffer (pH 8.0) containing 0.1M NaCl, 20% glycerol, 1mM DTT

0.5 mg/ml (determined by Bradford assay) (lot specific)

Can be stored at 4 degree C short term (1-2 weeks).
For long term storage, aliquot and store at -20 degree C or -70 degree C.
Avoid repeated freezing and thawing cycles.

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.

Polyglutamine binding protein 1, also known as PQBP1, is a transcription repressor that associates with polyglutamine tract-containing transcription regulators and causative genes for neurodegenerative disorders. PQBP-1 localizes to the nucleus and is present in neurons throughout the brain, with abundant levels in hippocampus, cerebellar cortex and olfactory bulb. PQBP-1 contains a WWP/WW domain that binds proline-rich motifs and a C2 domain that can influence Ca2+-dependent phospholipid signaling. Recombinant human PQBP1 protein, fused to His-tag at N-terminus, was expressed in E Coli and purified by using conventional chromatography techniques.

Metabolism

SDS-PAGE

33 kDa (289aa), confirmed by MALDI-TOF

polyglutamine binding protein 1

polyglutamine-binding protein 1

Polyglutamine-binding protein 1

Npw38??[Similar Products]

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]

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).

Waragai M., et al. (1999) Hum Mol Genet. 8:977-987.; Waragai M C., et al. (2000) Biochem Biophys Res. 273: 592-595.

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