SAB4200388

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

~1.5 mg/mL, affinity isolated antibody

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

Propiedades

• origen biológico: rabbit
• conjugado: unconjugated
• forma del anticuerpo: affinity isolated antibody
• tipo de anticuerpo: primary antibodies
• clon: polyclonal
• formulario: buffered aqueous solution
• mol peso: ~250 kDa
• reactividad de especies: human, mouse
• validación mejorada: recombinant expression; Learn more about Antibody Enhanced Validation
• concentración: ~1.5 mg/mL
• técnicas: 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
• Nº de acceso UniProt: O75923
• Condiciones de envío: dry ice
• temp. de almacenamiento: −20°C
• modificación del objetivo postraduccional: unmodified
• Información sobre el gen: human ... DYSF(8291) ; mouse ... Dysf(26903)

Descripción

Descripción general

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

Especificidad

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

Inmunógeno

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

Aplicación

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

• immunoblotting
• immunoprecipitation
• immunofluorescence

Acciones bioquímicas o fisiológicas

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.

Forma física

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

Almacenamiento y estabilidad

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.

Cláusula de descargo de responsabilidad

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.

Información de seguridad

• Código de clase de almacenamiento: 10 - Combustible liquids
• Punto de inflamabilidad (°F): Not applicable
• Punto de inflamabilidad (°C): Not applicable