SGCA, Polyclonal Antibody

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 Ig

Rabbit

This SGCA antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 239-266 amino acids from the Central region of human SGCA.

Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.

Vial Concentration: 0.29 (lot specific)

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

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.

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.

Signal Transduction

Western Blot (WB), ELISA (EIA)

WB~~1:1000

SGCA Antibody (Center) western blot analysis in 293 cell line lysates (35ug/lane).This demonstrates the SGCA antibody detected the SGCA protein (arrow).

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: Motility/polarity/chemotaxis; Membrane protein, integral

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

Mendell, J.R., et al. Ann. Neurol. 68(5):629-638(2010)
Fernandez, K., et al. Am. J. Pathol. 176(1):416-434(2010)
Klinge, L., et al. Neuromuscul. Disord. 18(12):934-941(2008)
Delgado-Olguin, P., et al. Biochim. Biophys. Acta 1779(1):74-80(2008)
Rafii, M.S., et al. J. Cell. Physiol. 209(2):439-447(2006)

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