bs-6479R-BF594

DATASHEET

Host: Rabbit

Target Protein: Myotilin

Immunogen Range: 121-200/498

Clonality: Polyclonal

Isotype: IgG

Entrez Gene: 9499

Source: KLH conjugated synthetic peptide derived from human Myotilin

Purification: Purified by Protein A.

Storage Buffer: Aqueous buffered solution containing 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

Storage: Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Background:

Myotilin, a sarcomeric protein that is encoded by the gene mapping to human chromosome 5q31, binds to a-actinin and is localized in the Z-line of myofibrils. Myotilin is expressed in skeletal and cardiac muscle, and it co-localizes with a-actinin in the sarcomeric I-bands where it directly interacts with a-actinin. Defects in the myotilin gene are reported to cause a form of autosomal dominant limb-girdle muscular dystrophy (LGMD). Symptoms of adult onset LGMD are progressive weakness of the hip and shoulder girdles as well as a distinctive dysarthric pattern of speech. The muscle of affected individuals with LGMD shows degeneration of myofibers, variations in fiber size, fiber splitting, centrally located myonuclei and an enhanced number of autophagic vesicles.

Conjugation: AbBy Fluor® 594

Excitation/ Emission: 590nm/617nm

Size: 100ul

Concentration: 1ug/ul

Applications: IF(IHC-P)(1:50-200)
IF(IHC-F)(1:50-200)
IF(ICC)(1:50-200)

Predicted Molecular Weight: 55

Predicted Cross Reactive Species: Human
Mouse
Rat
Dog
Cow
Pig
Horse

For research use only. Not intended for diagnostic or therapeutic use.