Mouse anti-Human PQBP1 Monoclonal Antibody | anti-PQBP1 antibody

PQBP1 (Polyglutamine-binding Protein 1, PQBP-1, 38 kDa Nuclear Protein Containing a WW Domain, Npw38, Polyglutamine Tract-binding Protein 1, NPW38, JM26)

Cat. Num. AAA6005286

• Gene Names: PQBP1; SHS; MRX55; MRXS3; MRXS8; NPW38; RENS1
• Reactivity: Human
• Applications: ELISA, Western Blot
• Purity: Affinity Purified; Purified by Protein A affinity chromatography.
• Synonyms: PQBP1; Monoclonal Antibody; PQBP1 (Polyglutamine-binding Protein 1; PQBP-1; 38 kDa Nuclear Protein Containing a WW Domain; Npw38; Polyglutamine Tract-binding Protein 1; NPW38; JM26); Anti -PQBP1 (Polyglutamine-binding Protein 1; anti-PQBP1 antibody

• Host: Mouse
• Reactivity: Human
• Clonality: Monoclonal
• Isotype: IgG2a,k
• Clone Number: 1A11
• Specificity: Recognizes human PQBP1.
• Purity/Purification: Affinity Purified; Purified by Protein A affinity chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2.
• Sequence: LAPYPKSKKAVSRKDEELDPMDPSSYSDAPRGTWSTGLPKRNEAKTGADTTAAGPLFQQRPYPSPGAVLRANAEASRTKQQD

• Applicable Applications for anti-PQBP1 antibody: ELISA (EL/EIA), Western Blot (WB)
• Application Notes: Suitable for use in ELISA and Western Blot.
• Immunogen: Partial recombinant corresponding to aa184-265 from PQBP1 (NP_005701) with GST tag. MW of the GST tag alone is 26kD.
• Preparation and Storage: 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 at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(Western Blot detection against Immunogen (34.76kD.)

Western Blot (WB)

(Western Blot analysis of PQBP1 expression in transfected 293T cell line by PQBP1 monoclonal antibodyLane 1: PQBP1 transfected lysate (30.5kD).|Lane 2: Non-transfected lysate.)

Testing Data

(Detection limit for recombinant GST tagged PQBP1 is 0.3ng/ml as a capture antibody.)

Related Product Information for anti-PQBP1 antibody

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.

Product Categories/Family for anti-PQBP1 antibody

Antibodies; Abs to Proteins

NCBI and Uniprot Product Information

• NCBI GI #: 269784739
• NCBI GeneID: 10084
• NCBI Accession #: NP_001161464.1
• NCBI GenBank Nucleotide #: NM_001167992.1
• UniProt Accession #: O60828; Q4VY25; Q4VY26; Q4VY27; Q4VY29; Q4VY30; Q4VY34; Q4VY35; Q4VY36; Q4VY37; Q4VY38; Q9GZP2
• Molecular Weight: 30,472 Da
• NCBI Official Full Name: polyglutamine-binding protein 1 isoform 6
• NCBI Official Synonym Full Names: polyglutamine binding protein 1
• NCBI Official Symbol: PQBP1
• NCBI Official Synonym Symbols: SHS; MRX55; MRXS3; MRXS8; NPW38; RENS1
• NCBI Protein Information: polyglutamine-binding protein 1; polyglutamine tract-binding protein 1; nuclear protein containing WW domain 38 kD; 38 kDa nuclear protein containing a WW domain
• UniProt Protein Name: Polyglutamine-binding protein 1
• Protein Family: Polyglutamine-binding protein
• UniProt Gene Name: PQBP1
• UniProt Synonym Gene Names: NPW38; PQBP-1; Npw38
• UniProt Entry Name: PQBP1_HUMAN

NCBI Description

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 mental retardation. Multiple alternatively spliced transcript variants that encode different protein isoforms have been described for this gene.[provided by RefSeq, Nov 2009]

Uniprot Description

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: nucleoplasm; stress granule; cytoplasm; nuclear speck; nucleus

Molecular Function: ribonucleoprotein binding; DNA binding; transcription coactivator activity

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

Disease: Renpenning Syndrome 1