Dystroglycan, beta

The dystroglycan complex is a membrane-spanning complex composed of two subunits, alpha- and beta-dystroglycan. Alpha-dystroglycan is a cell surface peripheral membrane protein which binds to the extracellular matrix (ECM), whereas beta-dystroglycan is an integral membrane protein which anchors alpha-dystroglycan to the cell membrane. The dystroglycan complex provides a tight link between the ECM and cell membrane. Dysfunction of the dystroglycan complex has commonly been implicated in the molecular pathogenesis of severe forms of hereditary neuromuscular diseases, including Duchenne muscular dystrophy, Fukuyama-type congenital muscular dystrophy and sarcoglycanopathy (1). Human dystroglycan is expressed in a variety of fetal and adult tissues. Data suggest that muscle and non-muscle isoforms of dystroglycan differ by carbohydrate moieties but not protein sequence. Therefore variable glycosylation of the conserved protein core might modulate laminin binding (2). beta-dystroglycan has been localized to microvilli structures in a number of cell types where it associates with the cytoskeletal adaptor ezrin, through which it is able to modulate the actin cytoskeleton and induce peripheral filopodia and microvilli (3).\n\nApplications: \nSuitable for use in Western Blotting. Other applications not tested.\n\nRecommended Dilution:\nWestern Blot: 1:1000-2000\nOptimal dilutions to be determined by the researcher.\n\nStorage and Stability:\nMay be stored at 4 degrees C for short-term only. For long-term storage, store at -20 degrees C. Aliquots are stable for at least 12 months at -20 degrees C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer. \n\nManufactured incorporating RabMAb(R) technology under Epitomics US patents, No 5,675,063 and 7,429,487, owned by Abcam.

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SPECIFICATIONS

Catalog Number

D9905-07

Size

100ul

Applications

WB

Hosts

Rabbit

Reactivities

Hum

Form

Supplied as a liquid in 50mM Tris-glycine, pH 7.4, 0.15M sodium chloride, 40% glycerol, 0.01% sodium azide, 0.05% BSA.

P Type

Mab

Purity

Supernatant

Isotype

IgG

References

1. Yamada H, et al. Hum Mol Genet 10(15):1563-9, 2001. 2. Ibraghimov-Beskrovnaya O, et al. Human Mol Genet 2(10) 1651-7,1993 3. Spence HJ, et al. Hum Mol Genet 13(15):1657-68, 2004.

Additional Info

Recognizes human beta Dystroglycan.