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C3orf39 抗体 (AA 40-378)

The 兔 多克隆 anti-C3orf39 antibody is suitable to detect C3orf39 in samples from 人, 小鼠 和 大鼠. It has been validated for WB 和 ELISA.
产品编号 ABIN7874697
发货至: 中国
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Quick Overview for C3orf39 抗体 (AA 40-378) (ABIN7874697)

抗原

See all C3orf39 (GTDC2) 抗体
C3orf39 (GTDC2) (Glycosyltransferase-Like Domain Containing 2 (GTDC2))

适用

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人, 小鼠, 大鼠

宿主

  • 32
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克隆类型

  • 34
多克隆

标记

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This C3orf39 antibody is un-conjugated

应用范围

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Western Blotting (WB), ELISA
  • 抗原表位

    • 2
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    AA 40-378

    原理

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

    纯化方法

    Immunogen affinity purified

    免疫原

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

    亚型

    IgG
  • 应用备注

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

    限制

    仅限研究用
  • 状态

    Lyophilized

    溶解方式

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

    缓冲液

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

    储存条件

    4 °C,-20 °C

    储存方法

    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.
  • 抗原

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

    别名

    POMGNT2

    背景

    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
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