Overview

  • Product name

    Anti-Alpha Dystroglycan antibody [2238]
    See all Alpha Dystroglycan primary antibodies
  • Description

    Mouse monoclonal [2238] to Alpha Dystroglycan
  • Host species

    Mouse
  • Specificity

    ab106110 is specific for a glycoepitope on brain bovine Alpha Dystroglycan, which is absent on Alpha Dystroglycan expressed in all other tissues.
  • Tested applications

    Suitable for: WB, IHC-Fr, ELISAmore details
  • Species reactivity

    Reacts with: Mouse, Rat, Rabbit, Cow, Human
  • Immunogen

    Other Immunogen Type corresponding to Cow Alpha Dystroglycan.

Properties

Applications

Our Abpromise guarantee covers the use of ab106110 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 1/50. Predicted molecular weight: 97 kDa. Recognizes Alpha-Dystroglycans as protein of ~130 kD, especially after enrichment of the lysates for dystroglycans.
IHC-Fr 1/50.
ELISA Use at an assay dependent concentration.

Target

  • Function

    The dystroglycan complex is involved in a number of processes including laminin and basement membrane assembly, sarcolemmal stability, cell survival, peripheral nerve myelination, nodal structure, cell migration, and epithelial polarization.
    Alpha-dystroglycan is an extracellular peripheral glycoprotein that acts as a receptor for both extracellular matrix proteins containing laminin-G domains, and for certain adenoviruses. Receptor for laminin-2 (LAMA2) and agrin in peripheral nerve Schwann cells. Also acts as a receptor for M.leprae in peripheral nerve Schwann cells but only in the presence of the G-domain of LAMA2, and for lymphocytic choriomeningitis virus, Old World Lassa fever virus, and clade C New World arenaviruses.
    Beta-dystroglycan is a transmembrane protein that plays important roles in connecting the extracellular matrix to the cytoskeleton. Acts as a cell adhesion receptor in both muscle and non-muscle tissues. Receptor for both DMD and UTRN and, through these interactions, scaffolds axin to the cytoskeleton. Also functions in cell adhesion-mediated signaling and implicated in cell polarity.
  • Tissue specificity

    Expressed in a variety of fetal and adult tissues. In epidermal tissue, located to the basement membrane. Also expressed in keratinocytes and fibroblasts.
  • Involvement in disease

    Defects in DAG1 are the cause of muscular dystrophy-dystroglycanopathy limb-girdle type C7 (MDDGC7) [MIM:613818]. An autosomal recessive muscular dystrophy showing onset in early childhood, and associated with mental retardation without structural brain anomalies. Note=MDDGC7 is caused by DAG1 mutations that interfere with normal post-translational processing, resulting in defective DAG1 glycosylation and impaired interactions with extracellular-matrix components. Other muscular dystrophy-dystroglycanopathies are caused by defects in enzymes involved in protein O-glycosylation.
  • Sequence similarities

    Contains 1 peptidase S72 domain.
  • Post-translational
    modifications

    O- and N-glycosylated. Alpha-dystroglycan is heavily O-glycosylated comprising of up to two thirds of its mass and the carbohydrate composition differs depending on tissue type. Mucin-type O-glycosylation is important for ligand binding activity. O-mannosylation of alpha-DAG1 is found in high abundance in both brain and muscle where the most abundant glycan is Sia-alpha-2-3-Gal-beta-1-4-Glc-NAc-beta-1-2-Man. In muscle, glycosylation on Thr-379 by a phosphorylated O-mannosyl glycan with the structure 2-(N-acetylamido)-2-deoxygalactosyl-beta-1,3-2-(N-acetylamido)-2-deoxyglucosyl-beta-1,4-6-phosphomannose is mediated by like-acetylglucosaminyltransferase (LARGE) protein and is required for laminin binding. O-mannosylation is also required for binding lymphocytic choriomeningitis virus, Old World Lassa fever virus, and clade C New World arenaviruses. The O-glycosyl hexose on Thr-367, Thr-369, Thr-372, Thr-381 and Thr-388 is probably mannose. O-glycosylated in the N-terminal region with a core 1 or possibly core 8 glycan. The beta subunit is N-glycosylated.
    Autolytic cleavage produces the alpha and beta subunits. In cutaneous cells, as well as in certain pathological conditions, shedding of beta-dystroglcan can occur releasing a peptide of about 30 kDa.
    SRC-mediated phosphorylation of the PPXY motif of the beta subunit recruits SH2 domain-containing proteins, but inhibits binding to WWW domain-containing proteins, DMD and UTRN. This phosphorylation also inhibits nuclear entry.
  • Cellular localization

    Secreted > extracellular space and Cell membrane. Cytoplasm > cytoskeleton. Nucleus > nucleoplasm. The monomeric form translocates to the nucleus via the action of importins and depends on RAN. Nuclear transport is inhibited by Tyr-892 phosphorylation. In skeletal muscle, this phosphorylated form locates to a vesicular internal membrane compartment. In peripheral nerves, localizes to the Schwann cell membrane. Colocalizes with ERM proteins in Schwann-cell microvilli.
  • Information by UniProt
  • Database links

  • Alternative names

    • 156DAG antibody
    • A3a antibody
    • AGRNR antibody
    • Alpha dystroglycan antibody
    • Alpha-DG antibody
    • Beta-DG antibody
    • Beta-dystroglycan antibody
    • DAG antibody
    • Dag1 antibody
    • DAG1_HUMAN antibody
    • Dystroglycan 1 (dystrophin associated glycoprotein 1) antibody
    • Dystroglycan antibody
    • Dystrophin associated glycoprotein 1 antibody
    • Dystrophin-associated glycoprotein 1 antibody
    • OTTHUMP00000210857 antibody
    • OTTHUMP00000210858 antibody
    see all

References

This product has been referenced in:

  • Barik A  et al. LRP4 is critical for neuromuscular junction maintenance. J Neurosci 34:13892-905 (2014). WB ; Mouse . Read more (PubMed: 25319686) »
  • Meilleur KG  et al. Clinical, pathologic, and mutational spectrum of dystroglycanopathy caused by LARGE mutations. J Neuropathol Exp Neurol 73:425-41 (2014). Read more (PubMed: 24709677) »
See all 3 Publications for this product

Customer reviews and Q&As

1-5 of 5 Abreviews or Q&A

Answer

Thank you for your email. We had 4 days of holidays, which is why your inquiry wasn't dealt in time. On the other hand Dr Tanya is out of office this week so I will be dealing with her inquiries.

I have read the whole protocol and information you have provided. It seems that you haven't fixed the samples; we always recommend customer fixing the samples in ice cold fixative e.g. acetone, methanol or ethanol for 15 minutes; this is necessary in IHC-FoFr which helps to avoid the antigen retrieval method.

The specificity note of ab106110 datasheet states, the antibody is only specific for a glycoepitope on brain bovine Alpha Dystroglycan, which is absent on Alpha Dystroglycan expressed in all other tissues. So we recommend using brain samples as positive control.

ab64568 was developed using muscle sample this ab could be perfect with your muscle sample.

I hope this information will be helpful. Should you have any question please do not hesitate to contact me.

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Answer

Thanks you for your email.

We have provided antibody ab106110 as per your order. It is clearly mentioned on the datasheet that ab64568 only detects the brain Alpha Dystroglycan because it is specific to a particular protein which is not expressed on other tissues so I would not expecting its use with muscle samples.

We do not change antibodies if they are incorrectly ordered and are used by customers. We can however provide exchanges in case of unopened and correctly stored antibodies. This means your order is not entitle for antibody exchange. However as your patients are waiting results so I am happy making an exception this time and will provide you ab64568 as replacement. Please note this antibody has not been tested in IHC-Fr yet so the experimental conditions has to be determined by the end user.

Please contact our Indian distributor from whom you purchased this product. They then need to contact us for agreed replacement.

I hope this info will be helpful. Should you have any question please do not hesitate to ask.

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Answer

Thank you for your inquiry.

I can confirm that we do not know of any publication using the above mentioned antibodies. I would like to reassure you that we do guarantee our product for all species and applications stated on the respective datasheet.

If you would like to use one of our products in a species or application that it has not been previously been tested in, you might be eligible for our testing discount program.

https://www.abcam.com/collaborationdiscount

For more information please see below:

At Abcam, providing quality service is just as important as providing quality products. Our Abpromise® ensures that you can trust our products to perform as stated on the datasheets, or we will offer a replacement, credit, or refund.

The Abcam Abpromise® guarantee:
- 100% Scientific and Customer Support for any product you buy from Abcam or one of our authorized distributors.
- We guarantee our products work in the tested species and applications stated on the datasheet.
- We will replace or refund products not performing as stated on the datasheet if reported within 6 months of purchase.
- We investigate any quality concerns raised by customers to ensure our catalog contains products that perform to the highest standards.

Please note these conditions to our Abpromise®:
- Protocol information must be provided in order for the claim to be validated.
- Antibodies tested in recombinant samples only are not guaranteed for use on endogenous samples.
- “Predicted to react” information on the datasheets is provided for reference only and these species are not guaranteed.
- Fast Track antibodies are covered for ELISA against the immunizing peptide only.

https://www.abcam.com/abpromise

I hope this information is helpful, but please do not hesitate to contact us with further questions.

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Question

Thanks for the prompt reply, i have filled up the proforma that you had sent and sending you with this mail, couldn't take the pic of the sections, but just to tell you, there was NO staining of the membrane of the muscle fibres as otherwise observed staining with the antibody on the positive sample.

Waiting for your reply

1) Abcam product code ab106110 (Anti-alpha dystroglycan antibody 2238)



2) Abcam order reference number or product batch number



3) Description of the problem No staining observed in the positive sample.



4) Sample preparation:

Species

Type of sample: Fresh frozen sections, perfusion fixed frozen sections, PFA/formalin fixed paraffin embedded sections, cells in culture, other: Fresh frozen sections (muscle biopsy)

Sample preparation

Positive control

Negative control



5) Fixation step NO

Yes/No

If yes: Fixative agent and concentration

Fixation time

Fixation temperature



6) Antigen retrieval method



7) Permeabilization method:

Did you do a permeabilization step (details please) or add permeabilizing agent in any dilution buffers?

Permeabilizing agent and concentration:





8) Blocking agent (eg BSA, serum…): PROTEIN BLOCK (AS GIVEN IN NOVOLINK KIT FROM NOVOCASTRA)

Concentration

Blocking time 5 MIN.

Blocking temperature ROOM TEMPERATURE



9) Endogenous peroxidases blocked? PEROXIDASE BLOCK(AS GIVEN IN NOVOLINK KIT FROM NOVOCASTRA)

Endogenous biotins blocked?



10) Primary antibody (If more than one was used, describe in “additional notes”) : ab106110

Concentration or dilution : (1:50) as given in the datasheet

Diluent buffer : TBS (tris-saline buffer)

Incubation time



11) Secondary antibody: NOVOLINK Polymer detection kit

Species:

Reacts against:

Concentration or dilution

Diluent buffer

Incubation time

Fluorochrome or enzyme conjugate



12) Washing after primary and secondary antibodies:

Buffer : TBS BUFFER (pH- 7.5)

Number of washes: THREE WASHES



13) Detection method NOVOLINK Polymer detection kit (DAB)



14) How many times have you run this staining? REGULARLY DOING WITH OTHER PRIMARY ANTIBODIES

Do you obtain the same results every time? DONE TWICE WITH ABCAM AB (106110), SAME NEGATIVE STAINING OBTAINED

What steps have you altered to try and optimize the use of this antibody? DIFF. DILUTIONS TRIED (1:20, 1:40)

Document attachment: Attaching images of your IHC is strongly recommended and can greatly speed up our investigation of your problem.

Thanks

















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Answer

Thank you for providing some useful information.

In order to be able to process your complaint, could you please confirm the batch number and the Abcam Order number (date of purchase)?

As our Abpromise indicates, in the event that a product is not functioning in the applications/species cited on the product data sheet (and the problem has been reported within 6 months of purchase) we will happily offer a credit note/refund to the value of the product purchased.

Alpha Dystroglycan is expressed in a variety of foetal and adult tissues. In epidermal tissue, located to the basement membrane, it is also expressed in keratinocytes and fibroblasts

Though you have kindly provided some details, it would be much appreciated if I could get some more information which would help me identify the source of the problem.

1) Samples:

Could you please specify the species of the sample? Is it human? Is the muscle biopsy from heart muscle, skeletal muscle or smooth muscle?

2) Secondary antibody/detection:

Does the secondary antibody recognizes the primary antibody? Could you please provide further information about the detection system specificity?

3) Positive control: any positive control used for checking the staining conditions?

Thank you for your understanding and co-operation in this matter. I look forward to hearing from you and hope to solve this problem as soon as possible.

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Answer

I am sorry to hear that you have been experiencing problems using this product in the application that you wish.

In order to assess the quality of our products I would ask that you complete a brief questionnaire relating to the application used. Often it is possible to make suggestions that may help resolve problems experienced using a particular product.

As our Abpromise indicates, in the event that a product is not functioning in the applications/species cited on the product data sheet (and the problem has been reported within 6 months of purchase) we will happily offer a credit note/refund to the value of the product purchased.

All our customer feedback, including complaints are monitored weekly by our in house technical support team. If a product is at fault the technical support team will consider removing the product from our catalogue in order to avoid future customer inconvenience.

Please could you provide some further details of the protocol used and complete the following form (attached as a word document). It would be much appreciated if you could attach an image to the response.

Thank you for your understanding and co-operation in this matter. I look forward to hearing from you soon and resolving this issue as soon as possible.

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