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

NA.41

Dysferlin is a 230 kDa transmembrane protein that belongs to the ferlin family of proteins including myoferlin and otoferlin and is homologous to the C. elegans fer-1 protein. It is expressed early during human development.

synthetic peptide corresponding to a sequence in the N-terminal region of human dysferlin, conjugated to KLH. The corresponding sequence is identical in human dysferlin isoforms 1-14 and identical in mouse dysferlin.

Anti-Dysferlin (N-terminal) antibody produced in rabbit has been used in several immunochemical techniques including immunoblotting, immunofluorescence and immunohistochemistry.

Dysferlin is implicated in membrane fusion events. It has also been involved in membrane repair processes, such as the ability to reseal the sarcolemma upon muscle injury. The integral membrane protein caveolin 3 have been shown to regulate the endocytosis of dysferlin. Dysferlin localization in the membrane and trafficking is impaired by mutations in caveolin-1 and 3, resulting in mistargeting and redistribution of dysferlin from the plasma membrane to the Golgi complex. Mutations in the dysferlin gene leads to the development of limb-girdle muscle dystrophy type 2B (LGMD2B), an autosomal recessive disorder and the related Miyoshi myopathy.

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

rabbit

200

unconjugated

affinity isolated antibody

primary antibodies

polyclonal

buffered aqueous solution

antigen ~250 kDa

rat, human, mouse

recombinant expression
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~1.0 mg/mL

dot blot: 0.1-0.2 μg/mL using extracts of HEK-293T cells over-expressing human dysferlin and 1-2 mg/mL using A10 cells.
immunohistochemistry: 20-30 μg/mL using methanol-acetone fixed frozen sections of mouse skeletal muscle.
indirect immunofluorescence: 5-10 μg/mL using differentiated C2C12 myoblasts.

O75923

dry ice

−20℃

human ... DYSF(8291)
mouse ... Dysf(26903)
rat ... Dysf(312492)

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