POMT1 anticorps (AA 224-747)

N° du produit ABIN7600716

Cet anticorps Lapin Polyclonal détecte spécifiquement POMT1 dans WB, ELISA et FACS. Il présente une réactivité envers Humain et Rat.

Aperçu rapide pour POMT1 anticorps (AA 224-747) (ABIN7600716)

Antigène: POMT1 (Protein-O-Mannosyltransferase 1 (POMT1))

Reactivité: Humain, Rat

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp POMT1 est non-conjugé

Application: Western Blotting (WB), ELISA, Flow Cytometry (FACS)

Détail du produit anti-POMT1 anticorps

Épitope: AA 224-747

Fonction: Anti-POMT1 Antibody Picoband®

Réactivité croisée (Details): No cross-reactivity with other proteins

Attributs du produit: Anti-POMT1 Antibody Picoband® (ABIN7600716). Tested in WB, Flow Cytometry, ELISA applications. This antibody reacts with Human, Rat. The brand Picoband indicates this is a premium antibody that guarantees superior quality, high affinity, and strong signals with minimal background in Western blot applications. Only our best-performing antibodies are designated as Picoband, ensuring unmatched performance.

Purification: Immunogen affinity purified.

Immunogène: E.coli-derived human POMT1 recombinant protein (Position: H224-H747). Human POMT1 shares 81.1% and 80.9% amino acid (aa) sequence identity with mouse and rat POMT1, respectively.

Isotype: IgG

Information d'application

Indications d'application: Western blot, 0.25-0.5 μg/mL, Human, Rat
Flow Cytometry (Fixed), 1-3 μg/1x10⁶ cells, Human
ELISA, 0.1-0.5 μg/mL
1. Balci, B., Uyanik, G., Dincer, P., Gross, C., Willer, T., Talim, B., Haliloglu, G., Kale, G., Hehr, U., Winkler, J., Topaloglu, H. An autosomal recessive limb girdle muscular dystrophy (LGMD2) with mild mental retardation is allelic to Walker-Warburg syndrome (WWS) caused by a mutation in the POMT1 gene. Neuromusc. Disord. 15: 271-275, 2005. 2. Bello, L., Melacini, P., Pezzani, R., D'Amico, A., Piva, L., Leonardi, E., Torella, A., Soraru, G., Palmieri, A., Smaniotto, G., Gavassini, B. F., Vianello, A., Nigro, V., Bertini, E., Angelini, C., Tosatto, S. C. E., Pegoraro, E. Cardiomyopathy in patients with POMT1-related congenital and limb-girdle muscular dystrophy. Europ. J. Hum. Genet. 20: 1234-1239, 2012. 3. Beltran-Valero de Bernabe, D., Currier, S., Steinbrecher, A., Celli, J., van Beusekom, E., van der Zwaag, B., Kayserili, H., Merlini, L., Chitayat, D., Dobyns, W. B., Cormand, B., Lehesjoki, A.-E., Cruces, J., Voit, T., Walsh, C. A., van Bokhoven, H., Brunner, H. G. Mutations in the O-mannosyltransferase gene POMT1 give rise to the severe neuronal migration disorder Walker-Warburg syndrome. Am. J. Hum. Genet. 71: 1033-1043, 2002.

Restrictions: For Research Use only

Stockage

Format: Lyophilized

Reconstitution: Adding 0.2 mL of distilled water will yield a concentration of 500 μg/mL.

Concentration: 500 μg/mL

Buffer: Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.

Stock: 4 °C,-20 °C

Stockage commentaire: At -20°C for one year from date of receipt. After reconstitution, at 4°C for one month.
It can also be aliquotted and stored frozen at -20°C for six months. Avoid repeated freezing and thawing.

Détails sur POMT1

Antigène: POMT1 (Protein-O-Mannosyltransferase 1 (POMT1))

Autre désignation: POMT1

Sujet:

Synonyms: POMT1, Protein O-mannosyl-transferase 1, EC 2.4.1.109, Dolichyl-phosphate-mannose--protein mannosyltransferase 1

Background: Protein O-mannosyl-transferase 1 is an enzyme that in humans is encoded by the POMT1 gene. The protein encoded by this gene is an O-mannosyltransferase that requires interaction with the product of the POMT2 gene for enzymatic function. The encoded protein is found in the membrane of the endoplasmic reticulum. Defects in this gene are a cause of Walker-Warburg syndrome (WWS) and limb-girdle muscular dystrophy type 2K (LGMD2K). Several transcript variants encoding different isoforms have been found for this gene.

Poids moléculaire: 75 kDa

ID gène: 10585

UniProt: Q9Y6A1