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Myotilin Polyclonal Antibody

Applications

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

Predicted Reactivity

  • Human
  • Mouse
  • Rat
  • Dog
  • Cow
  • Pig
  • Horse
Overview
Catalog # bs-6479R
Product Name Myotilin Polyclonal Antibody
Applications ELISA, IHC-P, IHC-F, IF(IHC-P), IF(IHC-F), IF(ICC)
Predicted Reactivity Human, Mouse, Rat, Dog, Cow, Pig, Horse
Specifications
Conjugation Unconjugated
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 0.01M TBS(pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
Storage Condition Shipped at 4°C. Store at -20°C for one year. 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
ELISA 1:500-1000
IHC-P 1:200-400
IHC-F 1:100-500
IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200