Anti-Dysferlin antibody produced in rabbit

Dysferlin is a transmembrane protein that belongs to the ferlin-1 family. It is mostly expressed in the early stages of human development. Dysferlin comprises a C-terminal transmembrane domain and a long N-terminal cytoplasmic region. Dysferlin gene is mapped to 2p13.2.

synthetic peptide corresponding to an internal sequence of human dysferlin (GeneID: 8291), conjugated to KLH.

Anti-Dysferlin antibody produced in rabbit may be used in:

• immunoblotting
• immunoprecipitation
• immunofluorescence

Anti-Dysferlin specifically recognizes human and mouse dysferlin.

Dysferlin localizes in the membrane and aids trafficking along with caveolin protein. The N-terminal region of dysferlin is essential for vesicular traffic and membrane fusion. Mutations in the dysferlin gene is implicated in the limb-girdle muscle dystrophy type 2B (LGMD2B), an autosomal recessive disorder and the related Miyoshi myopathy. It has been suggested to play a role in membrane fusion and repair processes, such as the ability to reseal the sarcolemma upon muscle injury. The integral membrane proteins caveolin-1 and -3 have been shown to regulate the endocytosis of dysferlin.4. Mutations in the caveolin-3 and 1 results in mistargeting and redistribution of dysferlin from the plasma membrane to the Golgi complex.

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

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 prolongedstorage, clarify the solution by centrifugation before use. Working dilutions should be discarded if not used within 12 hours.

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