SAB4200388

Anti-Dysferlin (N-terminal region) antibody produced in rabbit

~1.5 mg/mL, affinity isolated antibody

• Synonym(e): Anti-Dysferlin (N-terminal region) antibody produced in rabbit, Anti-DYSF, Anti-Limb girdle muscular dystrophy 2B
• UNSPSC-Code: 12352203
• NACRES: NA.41

Eigenschaften

• Biologische Quelle: rabbit
• Qualitätsniveau: 200
• Konjugat: unconjugated
• Antikörperform: affinity isolated antibody
• Antikörper-Produkttyp: primary antibodies
• Klon: polyclonal
• Form: buffered aqueous solution
• Mol-Gew.: ~250 kDa
• Speziesreaktivität: human, mouse
• Erweiterte Validierung: recombinant expression; Learn more about Antibody Enhanced Validation
• Konzentration: ~1.5 mg/mL
• Methode(n): immunocytochemistry: 7-14 μg/mL using differentiated C2C12 myoblasts; immunoprecipitation (IP): 5-10 μg using HEK-293T cells over-expressing human dysferlin; western blot: 0.5-1.0 μg/mL using HEK-293T cells over-expressing human dysferlin
• UniProt-Hinterlegungsnummer: O75923
• Versandbedingung: dry ice
• Lagertemp.: −20°C
• Posttranslationale Modifikation Target: unmodified
• Angaben zum Gen: human ... DYSF(8291) ; mouse ... Dysf(26903)

Beschreibung

Allgemeine Beschreibung

Dysferlin is a transmembrane protein, that belongs to the ferlin-1 family of proteins including myoferlin and otoferlin. It is homologous to the C. elegans fer-1 protein. Dysferlin is expressed in kidney cells, skeletal and cardiac muscle.

Dysferlin is encoded by the gene mapped to human chromosome 2p13.2

Spezifität

Anti-Dysferlin (N-terminal region) specifically recognizes human and mouse Dysferlin.

Immunogen

synthetic peptide corresponding to a sequence in the N-terminal region of human dysferlin (GeneID: 8291), conjugated to KLH.

Anwendung

Anti-Dysferlin (N-terminal region) antibody produced in rabbit has been used in:

• immunoblotting
• immunoprecipitation
• immunofluorescence

Biochem./physiol. Wirkung

Dysferlin is implicated in membrane fusion events. It plays a role in membrane repair processes, such as the ability to reseal the sarcolemma upon muscle injury. Dysferlin localization in the membrane and trafficking is impaired by mutations in caveolin-1 and -3. Mutations in the dysferlin gene are associated with limb-girdle muscle dystrophy type 2B (LGMD2B), distal anterior compartment myopathy and related Miyoshi myopathy.

Physikalische Form

solution in 0.01 M phos­phate buffered saline, pH 7.4, containing 15 mM sodium azide.

Lagerung und Haltbarkeit

For continuous use, store at 2-8 °C for up to one month. For extended storage, freeze in working aliquots. Repeated freezing and thawing, or storage in “frost-free” freezers,is not recommended. If slight turbidity occurs upon prolonged storage, clarify the solution by centrifugation before use. Working dilutions should be discarded if not used within 12 hours.

Haftungsausschluss

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

Sicherheitshinweise

• Lagerklassenschlüssel: 10 - Combustible liquids
• Flammpunkt (°F): Not applicable
• Flammpunkt (°C): Not applicable