C3orf39 antibody (AA 40-378)

Catalog No. ABIN7874697

The Rabbit Polyclonal anti-C3orf39 antibody is suitable to detect C3orf39 in samples from Human, Mouse and Rat. It has been validated for WB and ELISA.

Quick Overview for C3orf39 antibody (AA 40-378) (ABIN7874697)

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

Reactivity: Human, Mouse, Rat

Host: Rabbit

Clonality: Polyclonal

Conjugate: This C3orf39 antibody is un-conjugated

Application: Western Blotting (WB), ELISA

Product Details anti-C3orf39 Antibody

Binding Specificity: AA 40-378

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

Purification: Immunogen affinity purified

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

Isotype: IgG

Application Details

Application Notes: Optimal dilution of the POMGNT2 antibody should be determined by the researcher.

Restrictions: For Research Use only

Handling

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.

Storage: 4 °C,-20 °C

Storage Comment: 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.

Target Details for C3orf39

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

Alternative Name: POMGNT2

Background: 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