Mouse Alpha-Dystroglycan Monoclonal Antibody

Alpha-Dystroglycan, Bovine, mAb 2238

Cat. Num. AAA584240

• Reactivity: Reactivity: Bovine. Cross reactivity: Human, Mouse, Rabbit, Rat
• Applications: Western Blot
• Synonyms: Alpha-Dystroglycan; Monoclonal Antibody; Bovine; mAb 2238; Dystrophin-associated glycoprotein 1 (DAG1); anti-Alpha-Dystroglycan antibody

• Host: Mouse
• Reactivity: Reactivity: Bovine. Cross reactivity: Human, Mouse, Rabbit, Rat
• Clonality: Monoclonal
• Isotype: Mouse IgG2b
• Form/Format: 1 ml (100 ug/ml) 0.2 um filtered antibody solution in PBS, containing 0.1% bovine serum albumin and 0.02% sodium azide.; For unit size 0.5 mg or larger: PBS only (concentration< 0.5mg/ml)
• Sequence Length: 431

• Applicable Applications for anti-Alpha-Dystroglycan antibody: Immunoassay (IA), P, Western Blot (WB)
• Application Notes: Application Use: For immunohistology, and Western blotting, dilutions to be used depend on detection system applied. It is recommended that users test the reagent and determine their own optimal dilutions. The typical starting working dilution is 1:50.
• Preparation and Storage: Product should be stored at 4 degree C. Under recommended storage conditions, product is stable for one year.

Related Product Information for anti-Alpha-Dystroglycan antibody

Product Description: Monoclonal antibody 2238 recognizes a glycoepitope unique to brain alpha-dystroglycan. Alpha-dystroglycan (alpha-DG), also known as dystrophin-associated glycoprotein, is a laminin-binding protein of ~156 kDa (including glyco-groups). Alpha-DG is a component of the dystroglycan complex, which is involved in early development, morphogenesis and in the pathogenesis of muscular dystrophies. Alpha- and beta-DG are encoded by a single gene and are derived from a precursor polypeptide by posttranslational cleavage. Beta-DG is an integral membrane protein, whereas alpha-DG is membrane-associated through its noncovalent interaction with the extracellular domain of beta-DG. The alpha- and beta-DGs provide important physical linkages between components of basement membranes and cytoplasmic proteins that bind to the actin cytoskeleton. Alpha-DG is a heavily glycosylated, mucin-like protein anchored on the extracellular surface of the myotube, where it may provide linkage between the sarcolemma and extracellular matrix (ECM). Alpha-DG is expressed in a variety of fetal and adult tissues. Tissue-specific glycosylation modifies the laminin specificity of alpha-DG. The muscle and nonmuscle isoforms of dystroglycan differ by carbohydrate moieties but not protein sequence. Alpha-DG has been shown to colocalize with laminin in skeletal and cardiac muscle and a number of other cells including peripheral nerve, astrocytes, Purkinje neurons and kidney epithelium. Laminin-10/11 was shown to bind preferentially to brain alpha-DG. In Duchenne muscular dystrophy, the expression of alpha-DG is dramatically reduced leading to a loss of linkage between the sarcolemma and extracellular matrix, rendering muscle fibers more susceptible to necrosis. In the central nervous system, dystroglycan functions as a dual receptor for agrin and laminin-2 for instance in the Schwann cell membrane. Furthermore, defects in dystroglycan are central to the pathogenesis of structural and functional brain abnormalities seen in congenital muscular dystrophies (CMD). The monoclonal antibody 2238 is specific for a glycoepitope on brain bovine alpha-dystroglycan, which is absent on alpha-dystroglycan expressed in all other tissues.

References

McDearmon E et al; Brain alpha-dystroglycan displays unique glycoepitopes and preferential binding to laminin-10/11. FEBS Letters 2006, 580: 3381

NCBI and Uniprot Product Information

• NCBI GI #: 298683803
• Molecular Weight: 46,726 Da
• NCBI Official Full Name: alpha-dystroglycan, partial
• UniProt Protein Name: Alpha-dystroglycan
• UniProt Entry Name: D9IFY5_9MURI