PQBP1 anticorps (Middle Region)

N° du produit ABIN1108717

Cet anticorps Lapin Polyclonal détecte spécifiquement PQBP1 dans WB et IHC (p). Il présente une réactivité avec des échantillons de Humain, Souris, Rat, Boeuf (Vache) et Chien.

Aperçu rapide pour PQBP1 anticorps (Middle Region) (ABIN1108717)

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

Reactivité: Humain, Souris, Rat, Boeuf (Vache), Chien

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp PQBP1 est non-conjugé

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

Détail du produit anti-PQBP1 anticorps

Épitope: Middle Region

Séquence: PSCGLPYYWN ADREEGKERR HHRREELAPY PKSKKAVSRK DEELDPMDPS

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

Purification: Purified on Protein A affinity column

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

Isotype: IgG

Information d'application

Indications d'application: Optimal working dilution should be determined by the investigator.

Restrictions: For Research Use only

Stockage

Reconstitution: Add 100 μL of distilled water

Conseil sur la manipulation: Avoid repeated freezing and thawing.

Stock: -20 °C

Stockage commentaire: 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.

Date de péremption: 12 months

Détails sur PQBP1

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

Autre désignation: PQBP1

Sujet: 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

ID gène: 10084

NCBI Accession: NP_001027553

Pathways: Ribonucleoprotein Complex Subunit Organization