recombinant expression Learn more about Antibody Enhanced Validation
technique(s)
indirect immunofluorescence: 1:10,000-1:20,000 using HEK-293T cells overexpressing human dystrobrevin-a variant 6 western blot: 1:5,000-1:10,000 using lysates of HEK-293T cells overexpressing human dystrobrevin-a variant 6
Dystrobrevin-α (α-DB or DTNA) belongs to the dystrobrevin (DB) subfamily of the dystrophin-related-protein family.α-DB is a component of the dystrophin-glycoprotein complex (DGC) that is located at the muscle sarcolemma and forms a transmembrane link between the cytoskeleton and the basal lamina. The DB family includes two isoforms α- and β-DB encoded by the different genes. α-DB is also abundantly expressed in developing neurons and in the adult central nervous system (CNS). The DTNA gene is located on the human chromosome at 18q12.1.
Specificity
Anti-Dystrobrevin-α specifically recognizes human dystrobrevin-α.
Immunogen
synthetic peptide corresponding to a sequence at the C-terminal region of human dystrobrevin-a (DTNA), conjugated to KLH
Application
Anti-Dystrobrevin-α antibody produced in rabbit may be used in immunoblotting and immunofluorescence.
Biochem/physiol Actions
Dystrobrevin- α (α -DB) is involved in the formation and stability of synapses and the clustering of nicotinic acetylcholine receptors. Mutations in the DTNA gene are associated with congenital heart defects. Dystrobrevin is markedly reduced or absent in the sarcolemma of patients with Duchenne muscular dystrophy (DMD).
Physical form
solution in 0.01 M phosphate buffered saline, pH 7.4, containing 15 mM sodium azide
Storage and Stability
For continuous use, store at 2-8 °C for up to one month. For extended storage, freeze in working aliquots. Repeated freezing and thawing, or storage in “frost-free” freezers,is not recommended. If slight turbidity occurs upon prolonged storage, clarify the solution by centrifugation before use. Working dilutions should be discarded if not used within 12 hours.
Disclaimer
Unless otherwise stated in our catalog, our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
Prenatal diagnosis of de novo interstitial deletions involving 5q23. 1-q23. 3 and 18q12. 1-q12. 3 by array CGH using uncultured amniocytes in a pregnancy with fetal interrupted aortic arch and atrial septal defect
Chen C P, et al.
Gene, 531(2), 496-501 (2013)
Function and genetics of dystrophin and dystrophin-related proteins in muscle
Blake D J, et al.
Physiological Reviews (2002)
Dystrobrevins in muscle and non-muscle tissues
Rees M L J, et al.
Neuromuscular Disorders, 17(2), 123-134 (2007)
Dystrobrevin deficiency at the sarcolemma of patients with muscular dystrophy
Cellular and molecular life sciences : CMLS, 63(14), 1614-1631 (2006-05-20)
The dystrophin glycoprotein complex (DGC) is a multimeric protein assembly associated with either the X-linked cytoskeletal protein dystrophin or its autosomal homologue utrophin. In striated muscle cells, the DGC links the extracellular matrix to the actin cytoskeleton and mediates three
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