Background:

The protein encoded by DYSF (dysferlin) belongs to the ferlin family and is a skeletal muscle protein found associated with the sarcolemma. It is involved in muscle contraction and contains C2 domains that play a role in calcium-mediated membrane fusion events, suggesting that it may be involved in membrane regeneration and repair. In addition, the protein encoded by DYSF binds caveolin-3, a skeletal muscle membrane protein which is important in the formation of caveolae. Specific mutations in DYSF have been shown to cause autosomal recessive limb girdle muscular dystrophy type 2B (LGMD2B) as well as Miyoshi myopathy. Alternative splicing results in multiple transcript variants.

Alternative names:

DYSF; FER1L1; Dysferlin; Dystrophy-associated fer-1-like protein; Fer-1-like protein 1

Clonality:

Polyclonal

Reactivity:

Human,Mouse

Source:

Rabbit

Isotype:

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Immunogen:

Synthesized peptide derived from the C-terminal region of human Dysferlin. at AA rangle； 1950-2030

Concentration:

1mg/ml

Applications:

WB, IF, ELISA

Recommende dilutions:

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Storage:

PBS with 0.02% sodium azide and 50% glycerol pH 7.4. Store at -20°C. Avoid repeated freeze-thaw cycles.

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