SAB4200388

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

~1.5 mg/mL, affinity isolated antibody

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

Propriedades

• fonte biológica: rabbit
• Nível de qualidade: 200
• conjugado: unconjugated
• forma do anticorpo: affinity isolated antibody
• tipo de produto de anticorpo: primary antibodies
• clone: polyclonal
• Formulário: buffered aqueous solution
• peso molecular: ~250 kDa
• reatividade de espécies: human, mouse
• validação aprimorada: recombinant expression; Learn more about Antibody Enhanced Validation
• concentração: ~1.5 mg/mL
• técnica(s): 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 adesão UniProt: O75923
• Condições de expedição: dry ice
• temperatura de armazenamento: −20°C
• modificação pós-traducional do alvo: unmodified
• Informações sobre genes: human ... DYSF(8291) ; mouse ... Dysf(26903)

Descrição

Descrição geral

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

Especificidade

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

Imunogênio

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

Aplicação

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

• immunoblotting
• immunoprecipitation
• immunofluorescence

Ações bioquímicas/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.

Armazenamento e estabilidade

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.

Exoneração de responsabilidade

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.

Informações de segurança

• Código de classe de armazenamento: 10 - Combustible liquids
• Ponto de fulgor (°F): Not applicable
• Ponto de fulgor (°C): Not applicable