PQBP1 Antikörper (Middle Region)

Produktnummer ABIN1108717

Produktdetails anti-PQBP1 Antikörper

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

Bindungsspezifität: Middle Region

Reaktivität: Human, Maus, Ratte, Rind (Kuh), Hund

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser PQBP1 Antikörper ist unkonjugiert

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

Sequenz: PSCGLPYYWN ADREEGKERR HHRREELAPY PKSKKAVSRK DEELDPMDPS

Kreuzreaktivität (Details): Species reactivity (expected):Mouse, Rat, Bovine, DogSpecies reactivity (tested):Human

Aufreinigung: Purified on Protein A affinity column

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

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: Optimal working dilution should be determined by the investigator.

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Rekonstitution: Add 100 μL of distilled water

Handhabung: Avoid repeated freezing and thawing.

Lagerung: -20 °C

Informationen zur Lagerung: 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.

Haltbarkeit: 12 months

Details zu PQBP1

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

Andere Bezeichnung: PQBP1 (PQBP1 Produkte)

Hintergrund: 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

Gen-ID: 10084

NCBI Accession: NP_001027553

Pathways: Ribonucleoprotein Complex Subunit Organization