Overview

  • Product name

  • Description

    Rabbit polyclonal to GNPTAB
  • Host species

    Rabbit
  • Tested applications

    Suitable for: WBmore details
  • Species reactivity

    Reacts with: Human
    Predicted to work with: Mouse, Rat, Rabbit, Horse, Chicken, Guinea pig, Cow, Cat, Dog, Zebrafish
  • Immunogen

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

  • Positive control

    • HepG2 cell lysate

Properties

Applications

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.

Application Abreviews Notes
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.

Target

  • Function

    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.
  • Tissue specificity

    Expressed in the heart, whole brain, placenta, lung, liver, skeletal muscle, kidney and pancreas.
  • Involvement in disease

    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.
  • Sequence similarities

    Belongs to the stealth family.
    Contains 1 EF-hand domain.
    Contains 2 LNR (Lin/Notch) repeats.
  • Post-translational
    modifications

    The alpha- and beta-subunits appear to be generated by a proteolytic cleavage at the Lys-928-Asp-929 bond.
  • Cellular localization

    Golgi apparatus membrane.
  • Information by UniProt
  • Database links

  • Alternative names

    • EC=2.7.8.17 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

Images

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

    Predicted band size: 144 kDa



    Gel Concentration: 6-18%

References

ab108447 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

1-5 of 5 Abreviews or Q&A

Question
Answer

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, or if there is anything else I can help you with, please let me know.

Hope you have a great weekend.

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Question
Answer

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 replacement on the correct order.

Read More

Answer

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 even the date that the antibody was ordered?

I look forward to your reply and we will get a new vial of the antibody to you as soon as possible.

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Answer

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 (http://www.uniprot.org/uniprot/Q3T906). Depending on where the 144kD protein is cleaved into the two subunits, would determine which subunit will be detected. But, only one or the other subunit would be detected in this case, and not both.

Due to the issues that you are having with the antibody, and as I do not think any protocol advice that I could give would help resolve the issue, I would be happy to either replace or refund the cost of the antibody for you.

Please let me know how you would like to continue.

I look forward to your reply.

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Answer

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 the issue you are having with the antibody then I would be happy to either send a replacement antibody or to process a refund.

I just had two quick protocol questions about the HepG2 samples that you were using:

1 - Did you try loading 20ul of sample? As that is how much we recommend to load.

2 - Before loading the lysate, did you boil the samples?

I look forward to your reply and helping resolve this issue.

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