C3orf39 anticorps (AA 40-378)

N° du produit ABIN7874697

L’anticorps anti-C3orf39 Polyclonal Lapin est utilisé pour la détection de C3orf39 dans des échantillons de Humain, Souris et Rat. Il a été validé pour WB et ELISA.

Aperçu rapide pour C3orf39 anticorps (AA 40-378) (ABIN7874697)

Antigène: C3orf39 (GTDC2) (Glycosyltransferase-Like Domain Containing 2 (GTDC2))

Reactivité: Humain, Souris, Rat

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp C3orf39 est non-conjugé

Application: Western Blotting (WB), ELISA

Détail du produit anti-C3orf39 anticorps

Épitope: AA 40-378

Fonction: POMGNT2 Antibody / Protein O-linked mannose N-acetylglucosaminyltransferase 2

Purification: Immunogen affinity purified

Immunogène: E.coli-derived human POMGNT2 recombinant protein (Position: R40-K378) was used as the immunogen for the POMGNT2 antibody.

Isotype: IgG

Information d'application

Indications d'application: Optimal dilution of the POMGNT2 antibody should be determined by the researcher.

Restrictions: For Research Use only

Stockage

Format: Lyophilized

Reconstitution: Adding 0.2 mL of distilled water will yield a concentration of 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: After reconstitution, the POMGNT2 antibody can be stored for up to one month at 4oC. For long-term, aliquot and store at -20oC. Avoid repeated freezing and thawing.

Détails sur C3orf39

Antigène: C3orf39 (GTDC2) (Glycosyltransferase-Like Domain Containing 2 (GTDC2))

Autre désignation: POMGNT2

Sujet: POMGNT2 antibody detects Protein O-linked mannose N-acetylglucosaminyltransferase 2, an enzyme involved in the biosynthesis of O-mannosyl glycans essential for proper glycoprotein maturation. POMGNT2 catalyzes the addition of N-acetylglucosamine to O-linked mannose on target proteins, an essential step in forming the core M1 glycan structure. This modification plays a pivotal role in the development and stability of the nervous system and skeletal muscle by ensuring correct glycosylation of proteins such as alpha-dystroglycan. The POMGNT2 antibody is an important research tool for studying congenital muscular dystrophies and glycosylation disorders affecting the central nervous system and skeletal tissues.

POMGNT2 is encoded by the POMGNT2 gene located on human chromosome 3q26.1. The protein is localized primarily in the Golgi apparatus, where it functions as a type II membrane-bound glycosyltransferase. Structurally, POMGNT2 contains a luminal catalytic domain responsible for UDP-GlcNAc transfer and a short cytoplasmic N-terminal tail that directs subcellular localization. This enzyme works sequentially with other glycosyltransferases, including POMT1, POMT2, and POMGNT1, to synthesize complex O-mannosyl glycans critical for basement membrane integrity and synaptic architecture.

The POMGNT2 antibody is frequently used in biochemical and histological studies examining glycosylation pathways disrupted in congenital muscular dystrophy-dystroglycanopathy type B14 (MDDGB14). Loss-of-function mutations in POMGNT2 result in hypoglycosylated alpha-dystroglycan, leading to reduced laminin binding and severe developmental abnormalities, including brain malformations and muscular dystrophy. Western blot analysis with this antibody typically identifies a band near 70i 1/275 kDa corresponding to the mature enzyme, while immunofluorescence demonstrates punctate perinuclear staining consistent with Golgi localization.

Research employing POMGNT2 knockout models has revealed its necessity for neuronal migration, synaptic organization, and muscle fiber stability. In addition to its structural roles, glycosylation defects mediated by POMGNT2 deficiency affect signal transduction and receptor clustering in developing tissues. NSJ Bioreagents provides a validated POMGNT2 antibody for applications such as western blotting, immunofluorescence, and immunohistochemistry, supporting detailed exploration of glycosyltransferase function in normal and disease contexts.

UniProt: Q8NAT1