Antibody data
- Antibody Data
- Antigen structure
- References [0]
- Comments [0]
- Validations
- Western blot [1]
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- 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.
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Supportive validation
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- Bioworld Technology, Inc (provider)
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- Experimental details
- WesternBlot (WB) analysis of MYOT polyclonal antibody