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

This assay has high sensitivity and excellent specificity for detection of Human PQBP1. No significant cross-reactivity or interference between Human PQBP1 and analogues was observed.

Store at 2-8 degree C.

Select online data sheet information is drawn from bioinformatics databases, occasionally resulting in ambiguous or non-relevant product information. It is the responsibility of the customer to review, verify, and evaluate the information to make sure it matches their requirements before purchasing the kit. Our ELISA Kit assays are dynamic research tools and sometimes they may be updated and improved. If the format of this assay is important to you then please request the current manual or contact our technical support team with a presales inquiry before placing an order. We will confirm the current details of the assay. We cannot guarantee the sample manual posted online is the most current manual.

Small volumes of PQBP1 elisa kit 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.

MBS281202 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the Polyglutamine-binding protein 1 (PQBP1) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing PQBP1. The ELISA analytical biochemical technique of the MBS281202 kit is based on PQBP1 antibody-PQBP1 antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect PQBP1 antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, PQBP1. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

Principle of the Assay: This assay employs a two-site sandwich ELISA to quantitate PQBP1 in samples. An antibody specific for PQBP1 has been pre-coated onto a microplate. Standards and samples are pipetted into the wells and anyPQBP1 present is bound by the immobilized antibody. After removing any unbound substances, a biotin-conjugated antibody specific for PQBP1 is added to the wells. After washing, Streptavidin conjugated Horseradish Peroxidase (HRP) is added to the wells. Following a wash to remove any unbound avidin-enzyme reagent, a substrate solution is added to the wells and color develops in proportion to the amount of PQBP1 bound in the initial step. The color development is stopped and the intensity of the color is measured.

6,981 Da

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]

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.

Protein type: Transcription, coactivator/corepressor

Chromosomal Location of Human Ortholog: Xp11.23

Cellular Component: cytosol; neuronal ribonucleoprotein granule; nuclear speck; nucleoplasm; nucleus; stress granule

Molecular Function: DNA binding; double-stranded DNA binding; protein binding; ribonucleoprotein complex binding; transcription coactivator activity

Biological Process: activation of innate immune response; alternative mRNA splicing, via spliceosome; cellular response to exogenous dsRNA; defense response to virus; innate immune response; mRNA splicing, via spliceosome; neuron projection development; positive regulation of defense response to virus by host; positive regulation of interferon type I production; regulation of dendrite morphogenesis; regulation of RNA splicing; regulation of transcription, DNA-templated; transcription, DNA-dependent

Disease: Renpenning Syndrome 1

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