Rat Polyglutamine-binding protein 1 (PQBP1) ELISA kit; MRX55; MRXS3; MRXS8; NPW38; RENS1; SHS; nuclear protein containing WW domain 38 kD; polyglutamine binding protein 1

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

Store all reagents at 2-8 degree C

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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.

MBS9325279 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 MBS9325279 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(%).

30,529 Da

polyglutamine binding protein 1

polyglutamine-binding protein 1; PQBP-1; polyglutamine tract-binding protein 1

Polyglutamine-binding protein 1

PQBP-1??[Similar Products]

PQBP1_RAT

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

Cellular Component: nucleoplasm; centrosome; stress granule; cytoplasm; nuclear speck; nucleus

Molecular Function: protein C-terminus binding; ribonucleoprotein binding; protein binding

Biological Process: alternative nuclear mRNA splicing, via spliceosome; stress granule assembly; transcription, DNA-dependent; regulation of transcription, DNA-dependent; regulation of RNA splicing; regulation of dendrite morphogenesis; neurite development

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