Mouse monoclonal antibody [6C1 (4G2)] to DystroglycanDystroglycan 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. Alternative splicing results in multiple transcript variants all encoding the same protein.[provided by RefSeq, Apr 2010]
Immunogen
A synthetic peptide corresponding to amino acids 572-604 of human cranin (dystroglycan).
Key Feature
Clonality
Monoclonal
Isotype
IgM
Clone Number
6C1 (4G2)
Host Species
Mouse
Tested Applications
ELISAIHC-F
Immunohistology - Frozen: 1/1 - 1/50 Optimal dilution / concentration should be determined by the end user.:
Species Reactivity
HumanMouseChickenRatSheepXenopus
Concentration
1mg/ml
Purification
Affinity purified
Target Information
Gene Synonyms
MDDGC7 MDDGC9 AGRNR 156DAG DAG1 DAG A3A
Target
Cranin
Tissue Specificity
This antibody recognizes an extracellular region close to the C terminal end of the alpha subunit of human cranin, also known as dystroglycan.
Application Notes
Immunohistology - Frozen: 1/1 - 1/50 Optimal dilution / concentration should be determined by the end user.:
Additional Information
Form
Liquid
Storage Instructions
Store at -20°C only. Storage in frost-free freezers is not recommended. This product should be stored undiluted. Avoid repeated freezing and thawing as this may denature the antibody. Should this product contain a precipitate we recommend microcentrifugation before use.