Rabbit anti-Human SGCA Polyclonal Antibody | anti-SGCA antibody

SGCA, ID (SGCA, ADL, DAG2, Alpha-sarcoglycan, 50kD dystrophin-associated glycoprotein, Adhalin, Dystroglycan-2) (MaxLight 650)

Cat. Num. AAA6346831

• Gene Names: SGCA; A2; ADL; DAG2; DMDA2; 50-DAG; LGMD2D; SCARMD1; adhalin
• Reactivity: Human
• Applications: Western Blot
• Purity: Purified by Protein A and Peptide Affinity Chromatography.
• Synonyms: SGCA; Polyclonal Antibody; ID (SGCA; ADL; DAG2; Alpha-sarcoglycan; 50kD dystrophin-associated glycoprotein; Adhalin; Dystroglycan-2) (MaxLight 650); Dystroglycan-2; anti-SGCA antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes human SGCA.
• Purity/Purification: Purified by Protein A and Peptide Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with MaxLight650.

• Applicable Applications for anti-SGCA antibody: Western Blot (WB), FLISA
• Application Notes: Applications are based on unconjugated antibody.
• Immunogen: KLH-conjugated synthetic peptide mapping to a fragment of residues within amino acids 239-266 in the central region of human SGCA, Swiss/UniProt Accession: Q16586.
• Conjugate: MaxLight650
• Note: Preservative Free
• Preparation and Storage: Store product at 4 degree C in the dark. DO NOT FREEZE! Stable at 4 degree C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: MaxLight650 conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial prior to removing the cap.

Western Blot (WB)

(Western Blot analysis in 293 cell line lysates (35ug/lane) usingMBS647811. This demonstrates that MBS647811 detected the SGCA protein (arrow).)

Related Product Information for anti-SGCA antibody

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.

Product Categories/Family for anti-SGCA antibody

Antibodies; Carbohydrates; Glycoproteins

References

1. Mendell, J.R., et al. Ann. Neurol. 68(5):629-638(2010). 2. Fernandez, K., et al. Am. J. Pathol. 176(1):416-434(2010). 3. Klinge, L., et al. Neuromuscul. Disord. 18(12):934-941(2008). 4. Delgado-Olguin, P., et al. Biochim. Biophys. Acta 1779(1):74-80(2008). 5. Rafii, M.S., et al. J. Cell. Physiol. 209(2):439-447(2006).

NCBI and Uniprot Product Information

• NCBI GI #: 208973226
• NCBI GeneID: 6442
• NCBI Accession #: NP_001129169.1; NP_000014.1
• NCBI GenBank Nucleotide #: NM_001135697.1
• UniProt Accession #: Q16586; Q13710; Q13712; A6NEB8; A8K3K7
• Molecular Weight: 29,354 Da
• NCBI Official Full Name: alpha-sarcoglycan isoform 2
• NCBI Official Synonym Full Names: sarcoglycan, alpha (50kDa dystrophin-associated glycoprotein)
• NCBI Official Symbol: SGCA
• NCBI Official Synonym Symbols: A2; ADL; DAG2; DMDA2; 50-DAG; LGMD2D; SCARMD1; adhalin
• NCBI Protein Information: alpha-sarcoglycan; 50DAG; 50kD DAG; alpha-SG; dystroglycan-2; 50 kDa dystrophin-associated glycoprotein
• UniProt Protein Name: Alpha-sarcoglycan
• Protein Family: Alpha-sarcoglycan
• UniProt Gene Name: SGCA
• UniProt Synonym Gene Names: ADL; DAG2; Alpha-SG; 50DAG
• UniProt Entry Name: SGCA_HUMAN

NCBI Description

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]

Uniprot Description

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