ABCbiolab
SKU:ABCB01812
Dysferlin Polyclonal Antibody
Dysferlin Polyclonal Antibody
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Ab type
Primary antibody
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.
Reactivity
Human;Mouse
Clonality
Polyclonal
Host
Rabbit
Isotype
IgG
Immunogen
The antiserum was produced against synthesized peptide derived from human Dysferlin. AA range:1981-2030
Storage Stability
-20°C for one year
Concentration
1 mg/ml
MolecularWeight_Da
237295
synonyms
DYSF; FER1L1; Dysferlin; Dystrophy-associated fer-1-like protein; Fer-1-like protein 1
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