Anti-Dystrobrevin-a produced in rabbit, IgG fraction of antiserum Catalog Number SAB4200408 Product Description Reagent Anti-Dystrobrevin-a is produced in rabbit using as Supplied as a solution in 0.01 M phosphate buffered immunogen a synthetic peptide corresponding to a saline, pH 7.4, containing 15 mM sodium azide as a sequence at the C-terminal region of human preservative. dystrobrevin-a (DTNA) (GeneID 1837), conjugated to KLH. The corresponding sequence is identical in human Precautions and Disclaimer DTNA isoforms 2 and 6, and highly conserved (85% This product is for R&D use only, not for drug, sequence identity) in rat and mouse DTNA. Whole household, or other uses. Please consult the Material antiserum is purified using protein A immobilized on Safety Data Sheet for information regarding hazards agarose to provide the IgG fraction of antiserum. and safe handling practices. Anti-Dystrobrevin-a specifically recognizes human Storage/Stability dystrobrevin-a. The antibody can be used in several For continuous use, store at 2-8 °C for up to one month. immunochemical techniques including immunoblotting For extended storage, freeze in working aliquots. (~55 kDa) and immunofluorescence. Detection of the Repeated freezing and thawing, or storage in “frost- dystrobrevin-a band by immunoblotting is specifically free” freezers, is not recommended. If slight turbidity inhibited by the dystrobrevin-a immunizing peptide. occurs upon prolonged storage, clarify the solution by centrifugation before use. Working dilutions should be Dystrobrevin-a (also known as a-DB, DTNA, DRP3) discarded if not used within 12 hours. belongs to the dystrobrevin subfamily of the dystrophin Product Profile family. Dystrobrevin-a is a component of the dystrophin Immunoblotting: a working dilution of 1:5,000-1:10,000 glycoprotein complex (DGC) that is located at the is recommended using lysates of HEK-293T cells over- muscle sarcolemma and forms a transmembrane link between the cytoskeleton and the basal lamina.1,2 The expressing human dystrobrevin-a variant 6. DGC consists of dystrophin and several integral and peripheral membrane proteins, including dystroglycans, Immunofluorescence: a working dilution of sarcoglycans, syntrophins and dystrobrevins. DGC 1:10,000-1:20,000 is recommended using HEK-293T disruption is associated with various forms of muscular cells over-expressing human dystrobrevin-a variant 6. dystrophy, including Duchenne and Becker muscular dystrophies (DMD and BMD). In mammalian cells, the Note: In order to obtain the best results using various DB family includes two isoforms a- and b-DB encoded techniques and preparations, we recommend by different genes. In skeletal and cardiac muscle, determining the optimal working dilutions by titration. alternative splicing of a-DB mRNA gives rise to a least References five splice variants a-DB1-5 that differ in sequence and 2,3 1. Blake, D.J., et al., Physiol. Rev., 82, 291-329 (2002). tissue distribution. a-DB is also abundantly expressed 2. Haenggi, T., and Fritschy, J.M., Cell. Mol. Life Sci., in developing neurons and in the adult CNS, where it is 63, 1614-1631 (2006). thought to be involved in the formation and stability of 3. Rees, M.L.J., et al., Neuromuscular Disorders, 17, synapses and in the clustering of nicotinic acetylcholine 123-134 (2007). receptors.3,4 Mutations in the a-DB gene are associated 2 4. Lien, C.F., et al., Gene Expr. Patterns, 4, 583-593 with congenital heart defects. a-DB is markedly (2004). reduced or absent in the sarcolemma of patients with 1,5 5. Metzinger, L., et al., Human Mol. Genet., 6, 1185- DMD. 1191 (1997). ER,IDC,PHC 01/12-1 Ó2012 Sigma-Aldrich Co. LLC. All rights reserved. SIGMA-ALDRICH is a trademark of Sigma-Aldrich Co. LLC, registered in the US and other countries. Sigma brand products are sold through Sigma-Aldrich, Inc. Purchaser must determine the suitability of the product(s) for their particular use. Additional terms and conditions may apply. Please see product information on the Sigma-Aldrich website at www.sigmaaldrich.com and/or on the reverse side of the invoice or packing slip..