Anti-Dysferlin (N-terminal region) antibody produced in rabbit, approx. 1.5 mg/mL, affinity isolated antibody

Not available outside of the UK & Ireland.

Application

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

Biochem/physiol Actions

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.

Disclaimer

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.

General description

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

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.

Immunogen

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

Physical form

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

Specificity

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

Storage and Stability

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.