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Myotilin Polyclonal Antibody, RBITC Conjugated

Applications

  • IF(IHC-P)
  • IF(IHC-F)
  • IF(ICC)

Predicted Reactivity

  • Human
  • Mouse
  • Rat
  • Dog
  • Cow
  • Pig
  • Horse
Overview
Catalog # bs-6479R-RBITC
Product Name Myotilin Polyclonal Antibody, RBITC Conjugated
Applications IF(IHC-P), IF(IHC-F), IF(ICC)
Predicted Reactivity Human, Mouse, Rat, Dog, Cow, Pig, Horse
Specifications
Conjugation RBITC
Host Rabbit
Source KLH conjugated synthetic peptide derived from human Myotilin
Immunogen Range 121-200/498
Clonality Polyclonal
Isotype IgG
Concentration 1ug/ul
Purification Purified by Protein A.
Storage Buffer Aqueous buffered solution containing 0.01M TBS (pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage Condition Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
Target
Gene ID 9499
Subcellular location Cytoplasm, Cell membrane
Synonyms 57 kDa cytoskeletal protein; LGMD 1; LGMD1; Myofibrillar titin like Ig domains protein; Myofibrillar titin-like Ig domains protein; MYOT; MYOTI_HUMAN; Myotilin; Titin immunoglobulin domain protein; TTID; TTID protein.
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.
Application Dilution
IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200