Myotilin Antikörper (AA 121-200) (AbBy Fluor® 594)

Produktnummer ABIN2804433

Der Kaninchen Polyklonal Anti-Myotilin-Antikörper wurde für IF (cc) und IF (p) validiert. Er ist geeignet, Myotilin in Proben von Human zu detektieren.

Kurzübersicht für Myotilin Antikörper (AA 121-200) (AbBy Fluor® 594) (ABIN2804433)

Target: Myotilin (MYOT)

Reaktivität: Human

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser Myotilin Antikörper ist konjugiert mit AbBy Fluor® 594

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

Produktdetails anti-Myotilin Antikörper (AbBy Fluor® 594)

Bindungsspezifität: AA 121-200

Homologie: Human,Mouse,Rat,Dog,Cow,Pig,Horse

Aufreinigung: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human Myotilin

Isotyp: IgG

Anwendungsinformationen

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

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Konzentration: 1 μg/μL

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

Konservierungsmittel: ProClin

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

Lagerung: -20 °C

Informationen zur Lagerung: Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Haltbarkeit: 12 months

Details zu Myotilin

Target: Myotilin (MYOT)

Andere Bezeichnung: Myotilin

Hintergrund:

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.

Gen-ID: 9499