Anti-Beta Sarcoglycan/SGCB Antilichaam Picoband® fluoro488 Vervoeging

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.

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

SGCB

6443

Q16585

Human, Mouse, Rat

No cross-reactivity with other proteins

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.

Flow Cytometry

Immunogen affinity purified.

Liquid

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.

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

35 kDa

6

Sarcoglycan beta

Beta-sarcoglycan

Rabbit IgG

Each vial contains 50% glycerol, 0.9% NaCl, 0.2% Na2HPO4, 0.02% NaN3.