MYOT polyclonal, anti-human, mouse, rat
€388.00
In stock
SKU
BS8217
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 .
Alternative Name:
Myotilin, 57 kDa cytoskeletal protein, Myofibrillar titin-like Ig domains protein, Titin immunoglobulin domain protein, MYOT, TTID
Application Dilution: WB: 1:500 - 1:2000, IHC: 1:50 - 1:200, IF: 1:10 - 1:100
Specificity: MYOT polyclonal antibody detects endogenous levels of MYOT protein.
Immunogen:
Recombinant full length Human MYOT.
MW: ~ 55 kDa
Swis Prot.: Q9UBF9
Purification & Purity:
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).
Format:
1mg/ml in PBS with 0.1% Sodium Azide, 50% Glycerol.
Storage:
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
For research use only, not for use in diagnostic procedure.
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 .
Alternative Name:
Myotilin, 57 kDa cytoskeletal protein, Myofibrillar titin-like Ig domains protein, Titin immunoglobulin domain protein, MYOT, TTID
Application Dilution: WB: 1:500 - 1:2000, IHC: 1:50 - 1:200, IF: 1:10 - 1:100
Specificity: MYOT polyclonal antibody detects endogenous levels of MYOT protein.
Immunogen:
Recombinant full length Human MYOT.
MW: ~ 55 kDa
Swis Prot.: Q9UBF9
Purification & Purity:
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).
Format:
1mg/ml in PBS with 0.1% Sodium Azide, 50% Glycerol.
Storage:
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
For research use only, not for use in diagnostic procedure.
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