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.
Applications:Suitable for use in Western Blot, Immunofluorescence, ELISA.
Other applications not tested.
Recommended Dilution:Western Blot: 1:500-1:2000Immunofluorescence: 1:200-1:1000ELISA: 1:10,000Optimal dilutions to be determined by the researcher.
Storage and Stability:May be stored at 4°C for short-term only.
Aliquot to avoid repeated freezing and thawing.
Store at -20°C.
Aliquots are stable for 12 months after receipt.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size:30ul
Host:rabbit
Source Antibody:human
Grade:Affinity Purified
Purity:Purified by immunoaffinity chromatography.
Form:Supplied as a liquid in PBS, 0.5% BSA, 0.02% sodium azide, 50% glycerol.
Specificity:Recognizes endogenous levels of human Dysferlin. Species Crossreactivity: mouse
Isotype:IgG
Calc Applications Abbrev:E IF WB
Calc Crossreactivity:Hu Mo
Immunogen:Synthetic peptide corresponding to aa1950-2030 from the region of human Dysferlin at C-terminal.