Dystroglycan antibody
Quick Overview for Dystroglycan antibody (ABIN1107039)
Target
See all Dystroglycan (DAG1) AntibodiesReactivity
Host
Clonality
Conjugate
Application
Clone
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Specificity
- This antibody is specific for a glycoepitope on brain bovine alpha-dystroglycan, which is absent on alpha-dystroglycan expressed in all other tissues.
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Cross-Reactivity (Details)
- Species reactivity (tested):Human, mouse, rat, rabbit, bovine
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Purification
- Protein G purified
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Isotype
- IgG2b
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Application Notes
- Optimal working dilution should be determined by the investigator.
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Restrictions
- For Research Use only
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Concentration
- 0.1 mg/mL
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Buffer
- PBS, 0.02 % sodium azide, 0.1 % bovine serum albumin
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Preservative
- Sodium azide
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Precaution of Use
- This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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Storage
- 4 °C
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Storage Comment
- Store at 2 - 8 °C.
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- Dystroglycan (DAG1) (Dystroglycan 1 (DAG1))
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Alternative Name
- Dystroglycan
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Background
- 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).Synonyms: DAG1, Dystrophin-associated glycoprotein 1
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Gene ID
- 281439
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NCBI Accession
- NP_776587
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UniProt
- O18738
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Pathways
- Maintenance of Protein Location, Regulation of Carbohydrate Metabolic Process, Protein targeting to Nucleus
Target
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