Myotilin antibody (AA 121-200) (AbBy Fluor® 350)

Catalog No. ABIN1397525

Product Details anti-Myotilin Antibody (AbBy Fluor® 350)

Target: Myotilin (MYOT)

Binding Specificity: AA 121-200

Reactivity: Human

Host: Rabbit

Clonality: Polyclonal

Conjugate: This Myotilin antibody is conjugated to AbBy Fluor® 350

Application: Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))

Predicted Reactivity: Human,Mouse,Rat,Dog,Cow,Pig,Horse

Purification: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human Myotilin

Isotype: IgG

Application Details

Application Notes: IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200

Restrictions: For Research Use only

Handling

Format: Liquid

Concentration: 1 μg/μL

Buffer: Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.

Preservative: ProClin

Precaution of Use: This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.

Storage: -20 °C

Storage Comment: Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Expiry Date: 12 months

Target Details for Myotilin

Target: Myotilin (MYOT)

Alternative Name: Myotilin (MYOT Products)

Synonyms: LGMD1 antibody, LGMD1A antibody, MFM3 antibody, TTID antibody, TTOD antibody, 5530402I04Rik antibody, Ttid antibody, MYOT antibody, ttid antibody, myotilin antibody, MYOT antibody, Myot antibody, myot antibody

Background:

Synonyms: 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.

Background: 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.

Gene ID: 9499