Human DAG1 Recombinant Protein (RPPB3325)

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SKU:
RPPB3325
Product Type:
Recombinant Protein
Species:
Human
Uniprot:
Q14118

Description

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Product Name:Human DAG1 Recombinant Protein
Product Code:RPPB3325
Size:10µg
Species:Human
Target:DAG1
Synonyms:Dystroglycan, Dystrophin-associated glycoprotein 1, DAG1, A3a, DAG, AGRNR, 156DAG, MDDGC7, MDDGC9.
Source:Escherichia Coli
Physical Appearance:Filtered White lyophilized (freeze-dried) powder.
Formulation:DAG1 filtered (0.4 �m) and lyophilized from 0.5mg/ml in 0.05M phosphate buffer and 0.075M NaCl, pH 7.4.
Solubility:It is recommended to add 200�l deionized water to prepare a working stock solution of approximately 0.5mg/ml and let the lyophilized pellet dissolve completely. DAG1 is not sterile! Please filter the product by an appropriate sterile filter before using it in the cell culture.
Stability:Store lyophilized protein at -20°C. Aliquot the product after reconstitution to avoid repeated freezing/thawing cycles. Reconstituted protein can be stored at 4°C for a limited period of time; it does not show any change after two weeks at 4°C.
Purity:Greater than 90.0% as determined by SDS-PAGE.
Amino Acid Sequence:MKHHHHHHASHWPSEPSEAV RDWENQLEAS MHSVLSDLHE AVPTVVGIPD GTAVVGRSFR VTIPTDLIAS SGDIIKVSAA GKEALPSWLH WDSQSHTLEG LPLDTDKGVH YISVSATRLG ANGSHIPQTS SVFSIEVYPE DHSELQSVRT ASPDPGEVVS SACAADEPVT VLTVILDADL TKMTPKQRID LLHRMRSFSE VELHNMKLVP VVNNRLFDMS AFMAGPGNAK KVVENGALLS WKLGCSLNQN SVPDIHGVEA PAREGAMSAQ LGYPVVGWHI ANKKPPLPKR VRR

Dystroglycan 1 (DAG1) is a laminin binding component of the dystrophin-glycoprotein complex which provides a connection between the subsarcolemmal cytoskeleton and the extracellular matrix. The N-terminal domain of alpha-dystroglycan is secreted into the cerebrospinal fluid. The effect of DAG1 on the nervous system remains vague. The complete dystroglycan complex is expressed in a various tissues and has a role in processes such as laminin and basement membrane assembly, sarcolemmal stability, cell survival, peripheral nerve myelination, nodal structure, cell migration, and epithelial polarization. DAG1 is a candidate gene for the site of the mutation in autosomal recessive muscular dystrophies. The dramatic decrease of DAG1 in Duchenne muscular dystrophy leads to a loss of linkage between the sarcolemma and extracellular matrix, making muscle fibers more susceptible to necrosis.

DAG1 Human Recombinant produced in E.Coli is a single, non-glycosylated, polypeptide chain (a.a 30-312) containing 293 amino acids including a 10 a.a N-terminal His tag. The total molecular mass is 31.87kDa (calculated).

UniProt Protein Function: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.
UniProt Protein Details:

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

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 Code:Q14118
NCBI GenInfo Identifier:229462879
NCBI Gene ID:1605
NCBI Accession:Q14118.2
UniProt Secondary Accession:Q14118,Q969J9, A8K6M7,
UniProt Related Accession:Q14118
Molecular Weight:97,441 Da
NCBI Full Name:Dystroglycan
NCBI Synonym Full Names:dystroglycan 1 (dystrophin-associated glycoprotein 1)
NCBI Official Symbol:DAG1��
NCBI Official Synonym Symbols:A3a; DAG; AGRNR; 156DAG; MDDGC7; MDDGC9��
NCBI Protein Information:dystroglycan
UniProt Protein Name:Dystroglycan
UniProt Synonym Protein Names:Dystrophin-associated glycoprotein 1
Protein Family:Dystroglycan
UniProt Gene Name:DAG1��
UniProt Entry Name:DAG1_HUMAN
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