MYOT polyclonal antibody

Product Specifications

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) (1) . 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 .

CAS Number

9007-83-4

Structure Composition

Rabbit IgG, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2

Product Name Alternative

Myotilin; 57 kDa cytoskeletal protein; Myofibrillar titin-like Ig domains protein; Titin immunoglobulin domain protein; MYOT; TTID

Swiss Prot

Q9UBF9

Reactivity

Human, Mouse, Rat

Immunogen

Recombinant full length Human MYOT.

Conjugation

Unconjugated

Applications

WB IHC IF

Dilution

WB 1:500 - 1:2000 IHC 1:50 - 1:200 IF 1:10 - 1:100

Purity

The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE) .