Product Overview
Description
CLPP-00150542 is recombinant human DAG1 protein
Applications
ELISA, Mass Spectrometry, SDS-PAGE, Western Blot
Protein Length
Protein fragment
Nature
Recombinant Protein
Endotoxin Level
< 1.000 Eu/µg
Sequence
MKHHHHHHASHWPSEPSEAVRDWENQLEASMHSVLSDLHEAVPTVVGIPDGTAVVGRSFRVTIPTDLIASSGDIIKVSAAGKEALPSWLHWDSQSHTLEGLPLDTDKGVHYISVSATRLGANGSHIPQTSSVFSIEVYPEDHSELQSVRTASPDPGEVVSSACAADEPVTVLTVILDADLTKMTPKQRIDLLHRMRSFSEVELHNMKLVPVVNNRLFDMSAFMAGPGNAKKVVENGALLSWKLGCSLNQNSVPDIHGVEAPAREGAMSAQLGYPVVGWHIANKKPPLPKRVRR
Sequence Similarities
Contains 1 peptidase S72 domain.
Predicted Molecular Weight
32 kDa including tags
Target Information
Alternative Names
156DAG; A3a; Agrin receptor; AGRNR; Alpha-DG; alpha-DG-N; Beta-DG; Beta-dystroglycan; DAG; Dag1; DAG1_HUMAN; Dystroglycan; Dystroglycan 1 (dystrophin-associated glycoprotein 1); Dystroglycan, alpha; Dystrophin-associated glycoprotein 1; MDDGC7; MDDGC9; OTTHUMP00000210857; OTTHUMP00000210858
Protein Function
The dystroglycan complex is involved in a number of processes including laminin and basement membrane assembly, sarcolemmal stability, cell survival, peripheral nerve myelination, nodal structure, cell migration, and epithelial polarization.Alpha-dystroglycan is an extracellular peripheral glycoprotein that acts as a receptor for both extracellular matrix proteins containing laminin-G domains, and for certain adenoviruses. Receptor for laminin-2 (LAMA2) and agrin in peripheral nerve Schwann cells. Also acts as a receptor for M.leprae in peripheral nerve Schwann cells but only in the presence of the G-domain of LAMA2, and for lymphocytic choriomeningitis virus, Old World Lassa fever virus, and clade C New World arenaviruses.Beta-dystroglycan is a transmembrane protein that plays important roles in connecting the extracellular matrix to the cytoskeleton. Acts as a cell adhesion receptor in both muscle and non-muscle tissues. Receptor for both DMD and UTRN and, through these interactions, scaffolds axin to the cytoskeleton. Also functions in cell adhesion-mediated signaling and implicated in cell polarity.
Tissue Specificity
Expressed in a variety of fetal and adult tissues. In epidermal tissue, located to the basement membrane. Also expressed in keratinocytes and fibroblasts.
Involvement in Disease
Defects in DAG1 are the cause of muscular dystrophy-dystroglycanopathy limb-girdle type C7 (MDDGC7). An autosomal recessive muscular dystrophy showing onset in early childhood, and associated with mental retardation without structural brain anomalies. MDDGC7 is caused by DAG1 mutations that interfere with normal post-translational processing, resulting in defective DAG1 glycosylation and impaired interactions with extracellular-matrix components. Other muscular dystrophy-dystroglycanopathies are caused by defects in enzymes involved in protein O-glycosylation.
Shipping & Handling
Constituents
0.44% Sodium chloride, 99% Phosphate Buffer.
Shipping
Shipped at 4 °C.
