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Ordering Information

  • : 05-593
  • : 200 µL
  • : Please refer to the Certificate of Analysis for the lot-specific concentration.

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WB: Rabbit skeletal muscle lysate was probed with anti-α-Dystroglycan

Anti-α-Dystroglycan Antibody, clone IIH6C4

Species Reactivity Key Applications Host Format Antibody Type
H, R, M, Rb, Ca, Gp WB, Inhibition, IHC, IF Mouse Ascites Monoclonal Antibody
Description:
Anti-α-Dystroglycan Antibody, clone IIH6C4
Promotional Text:
Trade Name:
Upstate (Millipore)
Product Overview:
This product may be used for research purposes only. Diagnostic use of this product requires a license from the University of Iowa Research Foundation, 214 Technology Innovation Center, Iowa City, IA 52242
Specificity:
This antibody recognizes α-Dystroglycan/LARGE-glycan, Mr 156 kDa.
Molecular Weight:
156 kDa
Immunogen:
Rabbit skeletal muscle membrane preparation. Clone IIH6C4.
Clone:
IIH6C4
Isotype:
Background Information:
Dystroglycans are essential elements of the neuromuscular junction (NMJ). The gene for dystroglycan is expressed as a precursor protein that is post translationally cleaved into a 156 kDa extracellular peripheral membrane protein called alpha dystroglycan and a 43 kDa transmembrane protein, beta dystroglycan. The latter protein contains a PPxY motif that promotes binding to WW domain containing proteins, such as utrophin and dystrophin. Phosphorylation at tyrosine 892 within the PPxY motif may regulate c Src interactions with beta dystroglycan, as well as inhibit interactions with WW domain proteins. In skeletal muscle, beta dystroglycan is normally localized to the plasma membrane, however phosphorylation of Tyr892 leads to localization of beta dystroglycan to endosomal compartments along with c Src. Thus, phosphorylation at Tyr892 may have important roles in altering the localization of beta dystroglycan during NMJ formation.
Species Reactivity:
  • Human
  • Rat
  • Mouse
  • Rabbit
  • Canine
  • Guinea Pig
Species Reactivity Note:
Rabbit, human, mouse, rat, dog, and guinea pig.
Application Notes:
Inhibition of Laminin Binding to Dystroglycan: An independent lab has shown, in a nitrocellulose overlay experiment, that this antibody inhibits binding of 125I-laminin to dystroglycan (Ervasti, J., et al. (1993).

Western Blot Analysis: A previous lot of this antibody was used on mouse muscle tissue lysate 3 months after shRNA induction, and in littermate controls (ctrl) and LARGE-null negative control (myd) (Goddeeris, M., et al. 2013, Nature).

Immunofluorescence: A previous lot of this antibody was used to detect α-Dystroglycan /LARGE-glycan in mouse muscle tissue (Goddeeris, M., et al. 2013, Nature).
Control:
Rabbit skeletal muscle lysate.
Quality Assurance:
Routinely evaluated by western blot on rabbit skeletal muscle.

Western Blot Analysis:
A 1:1000-1:2000 dilution of this lot detected α-Dystroglycan/LARGE-glycan in rabbit skeletal muscle.
Note: The use of WGA purified protein results in significantly cleaner blots and immunoprecipitates.
Post-translational modification of dystroglycan causes band broadening.
Purification Method:
Unpurified
Presentation:
Mouse ascites, in PBS containing 0.05% sodium azide and 30% glycerol.
Liquid at -20ºC.
Storage Conditions:
Stable for 1 year at -20°C from date of receipt. For maximum recovery of product, centrifuge the vial prior to removing the cap.
UniProt Number:
Entrez Gene Number:
Gene Symbol:
  • DAG1
  • A3a
  • DAG
  • beta-dystroglycan
  • alpha-dystroglycan
  • AGRNR
  • 156DAG
Alternate Names:
  • Dystrophin-associated glycoprotein 1
  • dystroglycan 1
  • dystroglycan 1 (dystrophin-associated glycoprotein 1)
  • dystrophin-associated glycoprotein-1
  • LARGE-glycan
  • Large glycan
Usage Statement:
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
Key Applications:
  • Western Blotting
  • Inhibition
  • Immunohistochemistry
  • Immunofluorescence
Entrez Gene Summary:
Dystroglycan is a laminin binding component of the dystrophin-glycoprotein complex which provides a linkage between the subsarcolemmal cytoskeleton and the extracellular matrix. Dystroglycan 1 is a candidate gene for the site of the mutation in autosomal recessive muscular dystrophies. The dramatic reduction of dystroglycan 1 in Duchenne muscular dystrophy leads to a loss of linkage between the sarcolemma and extracellular matrix, rendering muscle fibers more susceptible to necrosis. Dystroglycan also functions as dual receptor for agrin and laminin-2 in the Schwann cell membrane. The muscle and nonmuscle isoforms of dystroglycan differ by carbohydrate moieties but not protein sequence.
UniProt Summary:
FUNCTION: SwissProt: Q14118 # Forms part of the dystrophin-associated protein complex (DAPC) which may link the cytoskeleton to the extracellular matrix. Alpha-dystroglycan functions as a laminin receptor. Binds to several types of arenaviruses. Is a target for the entry of Mycobacterium leprae into peripheral nerve Schwann cells.
SIZE: 895 amino acids; 97581 Da
SUBUNIT: Interacts with SGCD (By similarity). Interacts with AGR2 and AGR3.
SUBCELLULAR LOCATION: Alpha-dystroglycan: Secreted, extracellular space. & Beta-dystroglycan: Cell membrane; Single- pass type I membrane protein.
TISSUE SPECIFICITY: Expressed in a variety of fetal and adult tissues.
Brand Family:
Upstate
Product Name:
Anti-α-Dystroglycan Antibody, clone IIH6C4
Concentration:
Please refer to the Certificate of Analysis for the lot-specific concentration.
Antibody Type:
Monoclonal Antibody
Qty/Pk:
200 µL
Format:
Ascites
Host:
Mouse
Applications:
This Anti-α-Dystroglycan Antibody, clone IIH6C4 is validated for use in IH, FUNC, WB for the detection of α-Dystroglycan.

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