Anti-DAG1 / Dystroglycan Antibody (Internal) IHC-plus; DAG1; 156DAG; A3a; AGRNR; DAG; Dystroglycan; MDDGC7; Human DAG1; Dystroglycan

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

Polyclonal

Goat

Human DAG1 / Dystroglycan. Reported variants represent identical protein: NP_001159400.1, NP_004384.3

Immunoaffinity Purified

Tris-buffered saline, pH 7.3, 0.5% BSA, 0.02% sodium azide

0.5 mg/ml (lot specific)

Short term 4 degree C, long term aliquot and store at -20 degree C, avoid 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.

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

ELISA (1:128000), IHC-P (5 ug/ml), WB (0.2 - 0.6 ug/ml)
Usage: Peptide ELISA: antibody detection limit dilution 1:128000. Western blot: Approx 100kDa band observed in Human Skeletal Muscle lysates (calculated MW of 97.4kDa according to NP_004384.3). Recommended concentration: 0.2-0.6 ug/ml.

Anti-Dystroglycan antibody IHC of human heart. Immunohistochemistry of formalin-fixed, paraffin-embedded tissue after heat-induced antigen retrieval. Antibody concentration 5 ug/ml.

97,441 Da

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; Motility/polarity/chemotaxis; Cytoskeletal

Chromosomal Location of Human Ortholog: 3p21

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

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

Biological Process: nerve maturation; response to peptide hormone stimulus; 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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