PQBP1 antibody (Middle Region)

Catalog No. ABIN1108717

Product Details anti-PQBP1 Antibody

Target: PQBP1 (Polyglutamine Binding Protein 1 (PQBP1))

Binding Specificity: Middle Region

Reactivity: Human, Mouse, Rat, Cow, Dog

Host: Rabbit

Clonality: Polyclonal

Conjugate: This PQBP1 antibody is un-conjugated

Application: Western Blotting (WB), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p))

Sequence: PSCGLPYYWN ADREEGKERR HHRREELAPY PKSKKAVSRK DEELDPMDPS

Cross-Reactivity (Details): Species reactivity (expected):Mouse, Rat, Bovine, DogSpecies reactivity (tested):Human

Purification: Purified on Protein A affinity column

Immunogen: The immunogen for anti-PQBP1 antibody: synthetic peptide directed towards the middle region of human PQBP1.

Isotype: IgG

Application Details

Application Notes: Optimal working dilution should be determined by the investigator.

Restrictions: For Research Use only

Handling

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

Target Details for PQBP1

Target: PQBP1 (Polyglutamine Binding Protein 1 (PQBP1))

Alternative Name: PQBP1 (PQBP1 Products)

Synonyms: PQBP1 antibody, MRX55 antibody, MRXS3 antibody, MRXS8 antibody, NPW38 antibody, RENS1 antibody, SHS antibody, PQBP-1 antibody, Sfc2 antibody, npw38 antibody, polyglutamine binding protein 1 antibody, PQBP1 antibody, Pqbp1 antibody

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.Synonyms: 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

Pathways: Ribonucleoprotein Complex Subunit Organization