製品の概要

  • 製品名Anti-POMGNT1 antibody
    POMGNT1 一次抗体 製品一覧
  • 製品の詳細
    Rabbit polyclonal to POMGNT1
  • アプリケーション適用あり: WB, IHC-Pmore details
  • 種交差性
    交差種: Human
    交差が予測される動物種: Mouse, Rat, Cow, Pig
  • 免疫原

    Recombinant protein fragment with a sequence corresponding to a region within amino acids 98 and 343 of Human POMGNT1

  • ポジティブ・コントロール
    • HeLa, HepG2 cell lines. Paraffin-embedded Cal27 xenograft

法規制情報

製品の特性

  • 製品の状態Liquid
  • 保存方法Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
  • バッファーPreservative: 0.01% Thimerosal (merthiolate)
    Constituents: 10% Glycerol, 0.1M Tris, 0.1M Glycine, pH 7.0
  • Concentration information loading...
  • 精製度Immunogen affinity purified
  • ポリ/モノポリクローナル
  • アイソタイプIgG
  • 研究分野

関連製品

アプリケーション

Our Abpromise guarantee covers the use of ab96353 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

アプリケーション Abreviews 特記事項
WB 1/500 - 1/3000. Predicted molecular weight: 75 kDa.
IHC-P 1/100 - 1/500.

ターゲット情報

  • 機能Participates in O-mannosyl glycosylation. May be responsible for the synthesis of the GlcNAc(beta1-2)Man(alpha1-)O-Ser/Thr moiety on alpha-dystroglycan and other O-mannosylated proteins. Is specific for alpha linked terminal mannose and does not have MGAT3, MGAT4, MGAT5, MGAT7 or MGAT8 activity.
  • 組織特異性Constitutively expressed. An additional weaker band is also detected in spinal cord, lymph node, and trachea. Expressed especially in astrocytes. Also expressed in immature and mature neurons.
  • パスウェイProtein modification; protein glycosylation.
  • 関連疾患Defects in POMGNT1 are the cause of muscular dystrophy-dystroglycanopathy congenital with brain and eye anomalies type A3 (MDDGA3) [MIM:253280]. MDDGA3 is an autosomal recessive disorder characterized by congenital muscular dystrophy, ocular abnormalities, cobblestone lissencephaly and cerebellar hypoplasia. MDDGA3 patients present severe congenital myopia, congenital glaucoma, pallor of the optic disks, retinal hypoplasia, mental retardation, hydrocephalus, abnormal electroencephalograms, generalized muscle weakness and myoclonic jerks.
    Defects in POMGNT1 are the cause of muscular dystrophy-dystroglycanopathy congenital with mental retardation type B3 (MDDGB3) [MIM:613151]; also called muscular dystrophy congenital POMGNT1-related. MDDGB3 is an autosomal recessive disorder characterized by congenital muscular dystrophy associated with mental retardation and mild structural brain abnormalities. Clinical features include mental retardation, white matter changes, cerebellar cysts, pontine hypoplasia, myopia, optic atrophy, decreased alpha-dystroglycan on muscle biopsy and increased serum creatine kinase.
    Defects in POMGNT1 are the cause of muscular dystrophy-dystroglycanopathy limb-girdle type C3 (MDDGC3) [MIM:613157]; also called muscular dystrophy-dystroglycanopathy limb-girdle POMGNT1-related. MDDGC3 is a rare form of limb-girdle muscular dystrophy with normal cognition. Muscle biopsy shows dystrophic changes with variable staining for glycosylated alpha-dystroglycan.
  • 配列類似性Belongs to the glycosyltransferase 13 family.
  • ドメインAmino acid residues between 299-311 are important for both protein expression and enzymatic activity. The minimal catalytic domain is located between positions 299-651. Single amino acid substitutions in the stem domain from MEB patients abolished the activity of the membrane-bound form but not the soluble form. This suggests that the stem domain of the soluble form is unnecessary for activity, but that some amino acids play a crucial role in the membrane-bound form.
  • 細胞内局在Golgi apparatus membrane.
  • Information by UniProt
  • 参照データベース
  • 別名
    • 2-N-acetylglucosaminyltransferase 1 antibody
    • 2-N-acetylglucosaminyltransferase I.2 antibody
    • GnT I.2 antibody
    • GnTI.2 antibody
    • MEB antibody
    • MGAT 1.2 antibody
    • MGAT1.2 antibody
    • O linked mannose beta1 2 N acetylglucosaminyltransferase antibody
    • PMGT1_HUMAN antibody
    • POMGNT 1 antibody
    • POMGnT1 antibody
    • Protein O linked mannose beta 1 2 N acetylglucosaminyltransferase 1 antibody
    • Protein O linked mannose beta1 2 N acetylglucosaminyltransferase antibody
    • Protein O-linked-mannose beta-1 antibody
    • UDP GlcNAc antibody
    • UDP GlcNAc:alpha D mannoside beta 1 2 N acetylglucosaminyltransferase I.2 antibody
    • UDP-GlcNAc:alpha-D-mannoside beta-1 antibody
    see all

Anti-POMGNT1 antibody 画像

  • All lanes : Anti-POMGNT1 antibody (ab96353) at 1/1000 dilution

    Lane 1 : HeLa whole cell lysate
    Lane 2 : Hep G2 whole cell lysate

    Lysates/proteins at 30 µg per lane.


    Predicted band size : 75 kDa
  • ab96353, at 1/500, staining paraffin-embedded Cal27 xenograft by Immunohistochemistry.

Anti-POMGNT1 antibody (ab96353) 使用論文

ab96353 has not yet been referenced specifically in any publications.

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