Human Dystrophin Associated Glycoprotein 1 ELISA Kit | MyBioSource MBS095174 product information

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product summary

company name :

MyBioSource

product type :

ELISA/assay

product name :

Human Dystrophin Associated Glycoprotein 1 ELISA Kit

catalog :

MBS095174

quantity :

48-Strip-Wells

price :

470 USD

product information

catalog number :

MBS095174

products type :

ELISA Kit

products full name :

Human Dystrophin Associated Glycoprotein 1 ELISA Kit

products short name :

[Dystrophin Associated Glycoprotein 1]

other names :

[Dystroglycan; Dystroglycan; dystroglycan; dystroglycan 1 (dystrophin-associated glycoprotein 1); Dystrophin-associated glycoprotein 1]

products gene name :

[DAG1]

other gene names :

[DAG1; DAG1; A3a; DAG; AGRNR; 156DAG; MDDGC7; MDDGC9; Alpha-DG; Beta-DG]

uniprot entry name :

DAG1_HUMAN

reactivity :

Human

sequence length :

895

storage stability :

Store all reagents at 2-8 degree C

ncbi gi num :

229462879

ncbi acc num :

Q14118.2

uniprot acc num :

Q14118

ncbi mol weight :

97,441 Da

ncbi pathways :

Arrhythmogenic Right Ventricular Cardiomyopathy Pathway (672454); Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) Pathway (117293); Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) Pathway (116129); Dilated Cardiomyopathy Pathway (121494); Dilated Cardiomyopathy Pathway (121285); ECM Proteoglycans Pathway (833812); ECM-receptor Interaction Pathway (83068); ECM-receptor Interaction Pathway (479); Extracellular Matrix Organization Pathway (576262); Hypertrophic Cardiomyopathy (HCM) Pathway (114229)

ncbi summary :

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]

uniprot summary :

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: Cytoskeletal; Motility/polarity/chemotaxis; Membrane protein, integral. 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: tubulin binding; viral receptor activity; laminin-1 binding; protein binding; structural constituent of muscle; protein complex binding; SH2 domain binding; calcium ion binding; alpha-actinin binding; actin 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

size1 :

48-Strip-Wells

price1 :

470 USD

size2 :

96-Strip-Wells

price2 :

680

size3 :

5x96-Strip-Wells

price3 :

3100

size4 :

10x96-Strip-Wells

price4 :

6095