BS8217

antibody from Bioworld Technology, Inc
Targeting: MYOT LGMD1, LGMD1A, TTID
Provider product page for BS8217
Western blot
Immunohistochemistry

Antibody data

Product number
BS8217 - Provider product page
Provider
Bioworld Technology, Inc
Product name
MYOT polyclonal antibody
Antibody type
Polyclonal
Antigen
Recombinant full length Human MYOT.
Description
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 .
Reactivity
Human
Host
Rabbit
Isotype
IgG
Vial size
100ul
Concentration
1 mg/ml
Storage
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
MYOT protein structure - BS8217 shown in red.