Gene ID Number
1ug per 1ul
ALEXA FLUOR® 555
Human, Mouse, Rat
Purified by Protein A.
Alexa Fluor,ALEXA FLUOR 555
Conjugated Primary Antibodies
Rabbit (Oryctolagus cuniculus)
Anti-Myotilin PAb ALEXA FLUOR 555
This is a highly specific antibody against Myotilin.
Myotilin Polyclonal Antibody, ALEXA FLUOR 555 Conjugated
Due to limited amount of testing and knowledge, not every possible cross-reactivity is known.
This antibody was obtained by immunization of the host with KLH conjugated synthetic peptide derived from human Myotilin
Store this antibody in aqueous buffered solution containing 1% BSA, 50% glycerol and 0.09% sodium azide. Keep refrigerated at 2 to 8 degrees Celcius for up to one year.
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.
For facs or microscopy Alexa 1 conjugate.Very high photo stable ALEXA conjugate.If you buy Antibodies supplied by Bioss Primary Conjugated Antibodies. ALEXA FLUOR they should be stored frozen at - 24°C for long term storage and for short term at + 5°C.
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.