Anti -Polyglutamine (Polyglutamine Expansion Disease Marker)

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

Chromosome: X; NC_000086.7 (7894519..7899269, complement). Location: X A1.1; X 3.56 cM

Monoclonal

IgG1,k

2Q131

Mouse

Recognizes polyglutamine, homopolymeric glutamine stretches. The original immunogen was the general transcription factor TATA Box-binding protein (TBP) which contains a 38-Glns stretch.

Ascites
Ascites

Supplied as a liquid. No preservatives added.

May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

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.

Polyglutamine-containing proteins are recognized, notably those involved in several human neurodegenerative diseases caused by a CAG repeat expansion, like Huntington's disease and spinocerebellar ataxia type 2, 3 and 7. This antibody has been used to identify new neurodegenerative diseases caused by polyglutamine expansion and to help for cloning of the corresponding affected genes. This monoclonal antibody is also able to detect intracellular inclusions, which is a hallmark of such diseases. Shows greater detection of the pathological proteins that contain a polyglutamine expansion (37 glns) than the wild type proteins.

Antibodies; Abs to Disease Markers

ELISA (EL/EIA), Western Blot (WB), Immunoprecipitation (IP), Immunohistochemistry (IHC), Immunocytochemistry (ICC)

Suitable for use in ELISA, Western Blot, Immunohistochemistry, Immunocytochemistry and Immunoprecipitation.
Dilution: ELISA: 1:1000-1:20,000
Western Blot: 1:1000-1:20,000
Immunohistochemistry (Frozen & paraffin):1:1000-1:20,000
Immunocytochemistry: 1:1000-1:20,000 (transfected cells)
Immunoprecipitation: 1:1000-1:20,000

30,597 Da[Similar Products]

polyglutamine binding protein 1

polyglutamine-binding protein 1; scurfy candidate 2; polyglutamine tract-binding protein 1; 38 kDa nuclear protein containing a WW domain

Polyglutamine-binding protein 1

Npw38; PQBP-1; Npw38??[Similar Products]

PQBP1_MOUSE

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

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

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