Anti-Myotilin antibody (bs-6479R)

Anti-Myotilin antibody

DATASHEET

Host:Rabbit

Target Protein:Myotilin

IR:Immunogen Range:121-200/498

Clonality:Polyclonal

Isotype:IgG

Entrez Gene:9499

Swiss Prot:Q9UBF9

Source:KLH conjugated synthetic peptide derived from human Myotilin:121-200/498 

Purification:affinity purified by Protein A

Storage:0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Shipped at 4℃. Store at -20 °C for one year. 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.

Size:100ul

Concentration:1mg/ml

Applications:ELISA(1:5000-10000)
IHC-P(1:100-500)
IHC-F(1:100-500)
IF(1:50-200)

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

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