• 产品名称Anti-GNPTAB抗体
  • 描述
    兔多克隆抗体to GNPTAB
  • 经测试应用适用于: WBmore details
  • 种属反应性
    与反应: Human
    预测可用于: Mouse, Rat, Rabbit, Horse, Chicken, Guinea pig, Cow, Cat, Dog, Zebrafish
  • 免疫原

    Synthetic peptide corresponding to a region within N terminal amino acids 35-84 (FQFGEVVLEW SRDQYHVLFD SYRDNIAGKS FQNRLCLPMP IDVVYTWVNG) of Human GNPTAB (NP_077288)

  • 阳性对照
    • HepG2 cell lysate



Our Abpromise guarantee covers the use of ab108447 in the following tested applications.

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

应用 Ab评论 说明
WB Use a concentration of 1 µg/ml. Predicted molecular weight: 144 kDa. Good results were obtained when blocked with 5% non-fat dry milk in 0.05% PBS-T.


  • 功能Catalyzes the formation of mannose 6-phosphate (M6P) markers on high mannose type oligosaccharides in the Golgi apparatus. M6P residues are required to bind to the M6P receptors (MPR), which mediate the vesicular transport of lysosomal enzymes to the endosomal/prelysosomal compartment.
  • 组织特异性Expressed in the heart, whole brain, placenta, lung, liver, skeletal muscle, kidney and pancreas.
  • 疾病相关Defects in GNPTAB are the cause of mucolipidosis type II (MLII) [MIM:252500]; also known as inclusion cell disease or I-cell disease (ICD). MLII is a fatal, autosomal recessive, lysosomal storage disorder characterized by severe clinical and radiologic features, peculiar fibroblast inclusions, and no excessive mucopolysacchariduria. Congenital dislocation of the hip, thoracic deformities, hernia, and hyperplastic gums are evident soon after birth.
    Defects in GNPTAB are the cause of mucolipidosis type III complementation group A (MLIIIA) [MIM:252600]; also known as variant pseudo-Hurler polydystrophy. MLIIIA is an autosomal recessive disease of lysosomal enzyme targeting. Clinically MLIII is characterized by restricted joint mobility, skeletal dysplasia, and short stature. Mildly coarsened facial features and thickening of the skin have been described. Cardiac valvular disease and corneal clouding may also occur. Half of the reported patients show learning disabilities or mental retardation.
  • 序列相似性Belongs to the stealth family.
    Contains 1 EF-hand domain.
    Contains 2 LNR (Lin/Notch) repeats.
  • 翻译后修饰The alpha- and beta-subunits appear to be generated by a proteolytic cleavage at the Lys-928-Asp-929 bond.
  • 细胞定位Golgi apparatus membrane.
  • Information by UniProt
  • 数据库链接
  • 别名
    • EC= antibody
    • GlcNAc-1-phosphotransferase subunits alpha/beta antibody
    • GNPTA antibody
    • GNPTA_HUMAN antibody
    • Gnptab antibody
    • KIAA1208 antibody
    • N-acetylglucosamine-1-phosphotransferase subunit beta antibody
    • Stealth protein GNPTAB antibody
    • UDP-N-acetylglucosamine-1-phosphotransferase subunits alpha/beta antibody
    see all

Anti-GNPTAB antibody 图像

  • Anti-GNPTAB antibody (ab108447) at 1 µg/ml + HepG2 cell lysate at 10 µg

    Predicted band size : 144 kDa

Anti-GNPTAB antibody (ab108447)参考文献

ab108447 has not yet been referenced specifically in any publications.

Product Wall

Thanks for confirming that information.

You should receive the new vial of ab108447 next Tuesday (I set it to ship on Monday, instead of Saturday). For your records, the new order number is *.

If you have ay issues with the antibody, ...

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Thank you for your reply.

Unfortunately, that PO number does not match the one I found in our system. The one I have is PO# *and it was ordered 20th June 2012. Is this the correct one?

I just wish to make sure that I process the repla...

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Thank you for your reply.

I would be very happy to send you a new vial of ab108447. I think I have found your original order for this product, but to make sure, would you be able to provide me with either the Abcam order number, PO# number, or...

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Thanks for your reply.

Based on the sequence of the peptide used to raise this antibody, it is 100% homologous to both isoforms of the GNPTA protein (144kd and 56kD), according to the reviewed UniProt entry (

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Thank you for contacting Abcam.

I am sorry about the issues you have been having with ab108447 in western blot. The antibody is covered under our Abpromise for six months and is guaranteed to work in western blot on human. If we cannot resolve...

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