Alpha-sarcoglycan; Alpha-SG; 50 kDa dystrophin-associated glycoprotein; 50DAG; Adhalin; Dystroglycan-2; SGCA; ADL; DAG2

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit

Purified Rabbit Polyclonal Antibody (Pab)

0.01M PBS, pH 7.2, 0.1% Sodium azide, Glycerol 50%

1mg/ml (lot specific)

Store at -20 degree C.Stable for 12 months from date of receipt

Small volumes of anti-SGCA antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Component of the sarcoglycan complex, a subcomplex of the dystrophin-glycoprotein complex which forms a link between the F-actin cytoskeleton and the extracellular matrix.

Signal Transduction

Western Blot (WB), ELISA (EIA), Immunoprecipitation (IP)

WB~~ 1:1000

All lanes : Anti-Alpha-sarcoglycan Antibody at 1:1000 dilution
Lane 1: human skeletal musle lysates
Lane 2: human heart lysates

Lysates/proteins at 20 ug per lane.

Secondary
Goat Anti-Rabbit IgG, (H+L),Peroxidase conjugated at 1/10000 dilution

Predicted band size : 43 kDa

Blocking/Dilution buffer: 5% NFDM/TBST.

42875

sarcoglycan, alpha (50kDa dystrophin-associated glycoprotein)

alpha-sarcoglycan

Alpha-sarcoglycan

ADL; DAG2; Alpha-SG; 50DAG??[Similar Products]

SGCA_HUMAN

This gene encodes a component of the dystrophin-glycoprotein complex (DGC), which is critical to the stability of muscle fiber membranes and to the linking of the actin cytoskeleton to the extracellular matrix. Its expression is thought to be restricted to striated muscle. Mutations in this gene result in type 2D autosomal recessive limb-girdle muscular dystrophy. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Oct 2008]

SGCA: Component of the sarcoglycan complex, a subcomplex of the dystrophin-glycoprotein complex which forms a link between the F-actin cytoskeleton and the extracellular matrix. Defects in SGCA are the cause of limb-girdle muscular dystrophy type 2D (LGMD2D); also known as Duchenne- like muscular dystrophy autosomal recessive type 2 or severe childhood autosomal recessive muscular dystrophy (SCARMD). LGMD2D is an autosomal recessive degenerative myopathy characterized by progressive muscle wasting from early childhood with loss of independent ambulation by teenage years. Muscle biopsy shows necrosis, decreased immunostaining for alpha sarcoglycan, and adhalin deficiency. The phenotype is less severe than LGMD2C. Belongs to the sarcoglycan alpha/epsilon family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Membrane protein, integral; Motility/polarity/chemotaxis

Chromosomal Location of Human Ortholog: 17q21

Cellular Component: dystrophin-associated glycoprotein complex; cytoskeleton; cytoplasm; integral to membrane; sarcoglycan complex; intercellular junction; sarcolemma; lipid raft

Molecular Function: calcium ion binding

Biological Process: muscle development; muscle contraction

Disease: Muscular Dystrophy, Limb-girdle, Type 2d

Roberds S.L.,et al.Cell 78:625-633(1994).
McNally E.,et al.Proc. Natl. Acad. Sci. U.S.A. 91:9690-9694(1994).
Ota T.,et al.Nat. Genet. 36:40-45(2004).
Zody M.C.,et al.Nature 440:1045-1049(2006).
Mural R.J.,et al.Submitted (SEP-2005) to the EMBL/GenBank/DDBJ databases.

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