Polyglutamine Binding Protein 1 (PQBP1) (Middle Region) antibody

Details for Product No. ABIN1108717
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Antigen
Synonyms PQBP1, MRX55, MRXS3, MRXS8, NPW38, RENS1, SHS, PQBP-1, Sfc2, npw38
Epitope
Middle Region
(2), (2), (2), (1), (1), (1)
Reactivity
Cow (Bovine), Dog (Canine), Human, Mouse (Murine), Rat (Rattus)
(26), (17), (16), (14), (14), (12)
Host
Rabbit
(22), (4)
Clonality
Polyclonal
Conjugate
Un-conjugated
(1), (1), (1), (1), (1), (1), (1), (1), (1), (1), (1)
Application
Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Western Blotting (WB)
(17), (10), (6), (5), (2), (2), (2)
Pubmed 1 reference available
Quantity 0.1 mg
Shipping to United States (Change)
Availability Will be delivered in 6 to 8 Business Days
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Catalog No. ABIN1108717
335.50 $
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Immunogen The immunogen for anti-PQBP1 antibody: synthetic peptide directed towards the middle region of human PQBP1.
Sequence PSCGLPYYWN ADREEGKERR HHRREELAPY PKSKKAVSRK DEELDPMDPS
Isotype IgG
Purification Purified on Protein A affinity column
Alternative Name PQBP1
Background PQBP1 may suppress the ability of POU3F2 to transactivate the DRD1 gene in a POU3F2 dependent manner. It can activate transcription directly or via association with the transcription machinery. PQBP1 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.
Alternate names: 38 kDa nuclear protein containing a WW domain, NPW38, PQBP-1, Polyglutamine tract- binding protein 1, Polyglutamine-binding protein 1
Gene ID 10084
NCBI Accession NP_001027553
Research Area Signaling, Metabolism, Transcription Factors
Restrictions For Research Use only
Reconstitution Add 100 µL of distilled water
Handling Advice Avoid repeated freezing and thawing.
Storage -20 °C
Storage Comment Store the lyophised antibody at -20°C for up to one year. Store reconstitued antibody undiluted for one month or in aliquots at -20°C long term.
Expiry Date 12 months
Background publications Waragai, Lammers, Takeuchi et al.: "PQBP-1, a novel polyglutamine tract-binding protein, inhibits transcription activation by Brn-2 and affects cell survival." in: Human molecular genetics, Vol. 8, Issue 6, pp. 977-87, 1999 (PubMed).

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