Myotilin Antibody, Biotin Conjugated

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Catalog number: bs-6479R-Biotin
Price: 333 €
Supplier: Bioss Primary Conjugated Antibodies
Product name: Myotilin Antibody, Biotin Conjugated
Quantity: 0.1ml
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Long name: Biotin Conjugated, Myotilin Polyclonal Antibody
Category: Conjugated Primary Antibodies
Conjugation: Biotinylated
Host Organism: Rabbit (Oryctolagus cuniculus)
Target Antigen: Myotilin
Specificity: This is a highly specific antibody against Myotilin
Modification: Unmodified
Modification site: None
Clonality: Polyclonal
Clone: Polyclonal antibody
Concentration: 1ug per 1ul
Source: KLH conjugated synthetic peptide derived from human Myotilin
Gene ID number: 9499
Tested applications: IHC-P, WB
Recommended dilutions: IHC-P(1:100-500), WB(1:100-1000)
Crossreactivity: Mouse, Rat, Human
Crossreactive species details: not every possible cross-reactivity is known, Due to limited amount of testing and knowledge
Antigen background: Defects in the myotilin gene are reported to cause a form of autosomal dominant limb-girdle muscular dystrophy (LGMD), Myotilin is expressed in skeletal and cardiac muscle, 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, a sarcomeric protein that is encoded by the gene mapping to human chromosome 5q31, and it co-localizes with a-actinin in the sarcomeric I-bands where it directly interacts with a-actinin, binds to a-actinin and is localized in the Z-line of myofibrils, centrally located myonuclei and an enhanced number of autophagic vesicles, fiber splitting, variations in fiber size, Myotilin
Purification method: This antibody was purified via Protein A
Storage conditions: 50% glycerol and 0, Store refrigerated at 2 to 8 degrees Celcius for up to 1 year, 09% sodium azide, Keep the antibody in an aqueous buffered solution containing 1% BSA
Synonyms: LGMD 1, LGMD1, MYOT, MYOTI_HUMAN, Myofibrillar titin like Ig domains protein, Myofibrillar titin-like Ig domains protein, Myotilin, TTID, TTID protein, Titin immunoglobulin domain protein, 57 kDa cytoskeletal protein
Properties: Avidin and Streptavidin bind to the small biotin and are couple to HRP or AP for ELISA, Biotin conjugates can be detected by horseradish peroxidase, To break the streptavidin Biotin bond we suggest to use a 6 molar guanidine HCl solution with acidity of pH 1, alkaline phosphatase substrates or anti biotin conjugated antibodies, 6, C, C for long term storage and for short term at + 5°, If you buy Antibodies supplied by Bioss Primary Conjugated Antibodies they should be stored frozen at - 24°
Gene target: Myotilin Conjugated
Short name: Conjugated, Myotilin Antibody
Technique: antibodies against human proteins, antibodies for, antibody Conjugates, Antibody
Label: Biotin
Alternative name: biotinilated coupled, Myotilin (Antibody to)
Alternative technique: antibodies

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