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Anti-Beta Sarcoglycan/SGCB Antibody Picoband® HRP Conjugated

Product Specifications

Background

This gene encodes a member of the sarcoglycan family. Sarcoglycans are transmembrane components in the dystrophin-glycoprotein complex which help stabilize the muscle fiber membranes and link the muscle cytoskeleton to the extracellular matrix. Mutations in this gene have been associated with limb-girdle muscular dystrophy.

Synonyms

SGCB; Beta-sarcoglycan; Beta-SG; 43 kDa dystrophin-associated glycoprotein; 43DAG; A3b

Gene Name

SGCB

Gene ID

6443

UniProt

Q16585

Host

Rabbit

Reactivity

Human, Mouse, Rat

Cross Reactivity

No cross-reactivity with other proteins

Immunogen

E.coli-derived human Beta Sarcoglycan/SGCB recombinant protein (Position: E101-H318) . Human Beta Sarcoglycan/SGCB shares 95.4% amino acid (aa) sequence identity with mouse Beta Sarcoglycan/SGCB.

Clonality

Polyclonal

Applications

WB, IHC, ELISA

Purification

Immunogen affinity purified.

Form

Liquid

References & Citations

1. Barresi, R., Di Blasi, C., Negri, T., Brugnoni, R., Vitali, A., Felisari, G., Salandi, A., Daniel, S., Cornelio, F., Morandi, L., Mora, M. Disruption of heart sarcoglycan complex and severe cardiomyopathy caused by beta sarcoglycan mutations. J. Med. Genet. 37: 102-107, 2000. 2. Bonnemann, C. G., Modi, R., Noguchi, S., Mizuno, Y., Yoshida, M., Gussoni, E., McNally, E. M., Duggan, D. J., Angelini, C., Hoffman, E. P., Ozawa, E., Kunkel, L. M. Beta-sarcoglycan (A3b) mutations cause autosomal recessive muscular dystrophy with loss of the sarcoglycan complex. Nature Genet. 11: 266-273, 1995. Note: Erratum: Nature Genet. 12: 110 only, 1996. 3. Bonnemann, C. G., Passos-Bueno, M. R., McNally, E. M., Vainzof, M., Moreira, E. S., Marie, S. K., Pavanello, R. C. M., Noguchi, S., Ozawa, E., Zatz, M., Kunkel, L. M. Genomic screening for beta-sarcoglycan gene mutations: missense mutations may cause severe limb-girdle muscular dystrophy type 2E (LGMD 2E) . Hum. Molec. Genet. 5: 1953-1961, 1996.

Storage Conditions

At -20 ̊C for one year from date of receipt. Avoid repeated freezing and thawing.

Calculated Molecular Weight

35 kDa

Gene Name Synonym

Sarcoglycan beta

Protein Name

Beta-sarcoglycan

Isotype

Rabbit IgG

Contents

Each vial contains 50% glycerol, 0.9% NaCl, 0.2% Na2HPO4.

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