Dystroglycan; Dystrophin-associated glycoprotein 1; Alpha-dystroglycan; Alpha-DG; Beta-dystroglycan; Beta-DG; DAG1

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit Ig

Rabbit

This DAG1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 718-747 amino acids from the C-terminal region of human DAG1.

Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.

Vial Concentration: 0.5 (lot specific)

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Small volumes of anti-DAG1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

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.
Alternative splicing results in multiple transcript variants all
encoding the same protein.

Neuroscience; Signal Transduction

Western Blot (WB), ELISA (EIA), Immunohistochemistry (IHC)

WB~~1:1000

DAG1 Antibody (C-term) western blot analysis in mouse NIH-3T3 cell line lysates (35ug/lane).This demonstrates the DAG1 antibody detected the DAG1 protein (arrow).

DAG1 Antibody (C-term) (MBS9205082)immunohistochemistry analysis in formalin fixed and paraffin embedded human skeletal muscle followed by peroxidase conjugation of the secondary antibody and DAB staining.This data demonstrates the use of DAG1 Antibody (C-term) for immunohistochemistry. Clinical relevance has not been evaluated.

97441

dystroglycan 1 (dystrophin-associated glycoprotein 1)

dystroglycan

Dystroglycan

Alpha-DG; Beta-DG??[Similar Products]

DAG1_HUMAN

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. Alternative splicing results in multiple transcript variants all encoding the same protein.[provided by RefSeq, Apr 2010]

DAG1: a cytoskeletal protein that functions as a laminin receptor. Provides a linkage between the subsarcolemmal cytoskeleton and the extracellular matrix. May be involved in autosomal recessive muscular dystrophies. Its dramatic reduction 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. Binds to several types of arenaviruses. Is a target for the entry of Mycobacterium leprae into peripheral nerve Schwann cells. The muscle and nonmuscle isoforms of dystroglycan differ by carbohydrate moieties but not protein sequence.

Protein type: Membrane protein, integral; Cytoskeletal; Motility/polarity/chemotaxis

Chromosomal Location of Human Ortholog: 3p21

Cellular Component: dystroglycan complex; extracellular space; costamere; focal adhesion; basolateral plasma membrane; extracellular region; integral to membrane; lipid raft; nucleoplasm; dystrophin-associated glycoprotein complex; postsynaptic membrane; cytoskeleton; cell-cell adherens junction; lamellipodium; cytoplasm; plasma membrane; basement membrane; sarcolemma; filopodium

Molecular Function: tubulin binding; laminin-1 binding; viral receptor activity; protein binding; structural constituent of muscle; protein complex binding; calcium ion binding; SH2 domain binding; alpha-actinin binding; actin binding; vinculin binding

Biological Process: response to peptide hormone stimulus; nerve maturation; entry of virus into host cell; extracellular matrix organization and biogenesis; negative regulation of protein kinase B signaling cascade; cytoskeletal anchoring; negative regulation of MAPKKK cascade; membrane protein ectodomain proteolysis; NLS-bearing substrate import into nucleus; myelination in the peripheral nervous system; virus-host interaction; morphogenesis of an epithelial sheet; calcium-dependent cell-matrix adhesion; negative regulation of cell migration

Disease: Muscular Dystrophy-dystroglycanopathy (limb-girdle), Type C, 9; Muscular Dystrophy-dystroglycanopathy (congenital With Brain And Eye Anomalies), Type A, 9

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Masaki, T., et al. J. Biomed. Biotechnol. 2010, 740403 (2010) :

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