Anti-MYOT Antibody Picoband® fluoro550 Vervoeging

Myotilin is a protein that in humans is encoded by the MYOT gene. This gene encodes a cystoskeletal protein which plays a significant role in the stability of thin filaments during muscle contraction. This protein binds F-actin, crosslinks actin filaments, and prevents latrunculin A-induced filament disassembly. Mutations in this gene have been associated with limb-girdle muscular dystrophy and myofibrillar myopathies. Several alternatively spliced transcript variants of this gene have been described, but the full-length nature of some of these variants has not been determined.

MYOT

9499

Q9UBF9

Human, Mouse, Rat

E.coli-derived human MYOT recombinant protein (Position: H9-E494) . Human MYOT shares 88.9% amino acid (aa) sequence identity with mouse MYOT.

Flow Cytometry

Immunogen affinity purified.

Liquid

1. Foroud, T., Pankratz, N., Batchman, A. P., Pauciulo, M. W., Vidal, R., Miravalle, L., Goebel, H. H., Cushman, L. J., Azzarelli, B., Horak, H., Farlow, M., Nichols, W. C. A mutation in myotilin causes spheroid body myopathy. Neurology 65: 1936-1940, 2005. 2. Goebel, H. H., Muller, J., Gillen, H. W., Merritt, A. D. Autosomal dominant 'spheroid body myopathy'. Muscle Nerve 1: 14-26, 1978. 3. Hauser, M. A., Conde, C. B., Kowaljow, V., Zeppa, G., Taratuto, A. L., Torian, U. M., Vance, J., Pericak-Vance, M. A., Speer, M. C., Rosa, A. L. Myotilin mutation found in second pedigree with LGMD1A. Am. J. Hum. Genet. 71: 1428-1432, 2002.

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

6

IgG

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