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) .
Modification
Unmodification
Molecular Weight
~ 55 kDa
Storage Conditions
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
Notes
For research use only, not for use in diagnostic procedure.
Specificity
MYOT polyclonal antibody detects endogenous levels of MYOT protein.
Applications Notes
WesternBlot (WB) analysis of MYOT polyclonal antibody
Host or Source
Rabbit
Available Sizes
Curated Selection
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