Myotilin Polyclonal Antibody, AbBy Fluor-555 Conjugated
Catalogue Number: BS-6479R-BF555-BSS
| Manufacturer: | Bioss Inc |
| Type: | Polyclonal Primary Antibody - Conjugated |
| Alias: | 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. |
| Shipping Condition: | Blue Ice |
| Unit(s): | 100 ul |
| Host name: | Rabbit |
| Clone: | |
| Isotype: | IgG |
| Immunogen: | KLH conjugated synthetic peptide derived from human Myotilin |
| Application: | ICC, IF, IHC-P, IHC-Fr |
Description
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). 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.
Additional Text
Gene Name
MYOT
Gene ID
9499
Purification
Protein A purified
Concentration
1 ug/ul
Antibody Clonality
Polyclonal
Modification
Unmodified
Storage Note
Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
Excitation
553nm/568nm
BS-6479R-BF555 Datasheet
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