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Anti-Dysferlin antibody (bs-2429R)

Anti-Dysferlin antibody (bs-2429R)

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Anti-Dysferlin antibody 

英文名称Dysferlin
中文名称Dysferlin蛋白抗体
别    名DMAT; DYSF; Dysferlin (Dystrophy associated fer 1 like protein) (Fer 1 like protein 1); Dysferlin limb girdle muscular dystrophy 2B (autosomal recessive); Dysferlin limb girdle muscular dystrophy 2B; Dystrophy associated fer 1 like 1; Dystrophy associated fer 1 like protein; Dystrophy associated fer1 like 1; Dystrophy associated fer1 like protein; Fer 1 like protein 1; Fer1 like protein 1; FER1L1; FLJ00175; FLJ90168; LGMD 2B; LGMD2B; Limb girdle muscular dystrophy 2B (autosomal recessive) ; Limb girdle muscular dystrophy 2B; Miyoshi myopathy; MM; DYSF_HUMAN.

DATASHEET

Host:Rabbit

Target Protein:Dysferlin

IR:Immunogen Range:1901-2119/2119

Clonality:Polyclonal

Isotype:IgG

Entrez Gene:8291

Swiss Prot:O75923

Source:KLH conjugated synthetic peptide derived from human Dysferlin:1901-2119/2119 

Purification:affinity purified by Protein A

Storage:0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.

Background:The protein encoded by this gene 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 this gene binds caveolin-3, a skeletal muscle membrane protein which is important in the formation of caveolae. Specific mutations in this gene 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. [provided by RefSeq, Aug 2008].

Size:100ul

Concentration:1mg/ml

Applications:ELISA(1:5000-10000)
Flow-Cyt(3ug/Test)

Cross Reactive Species:Human
Mouse
Rat
Dog
Pig
Cow
Horse
Rabbit
Guinea Pig
.

For research use only. Not intended for diagnostic or therapeutic use.

VALIDATION IMAGES

Blank control:U937.
Primary Antibody (green line): Rabbit Anti-Dysferlin antibody (bs-2429R)
Dilution: 2μg /10^6 cells;
Isotype Control Antibody (orange line): Rabbit IgG .
Secondary Antibody : Goat anti-rabbit IgG-PE
Dilution: 1μg /test.
Protocol
The cells wereincubated in 5%BSA to block non-specific protein-protein interactions for 30 min at room temperature .Cells stained with Primary Antibody for 30 min at room temperature. The secondary antibody used for 40 min at room temperature. Acquisition of 20,000 events was performed.

Blank control (Black line): Mouse kidney (Black).
Primary Antibody (green line): Rabbit Anti-Dysferlin antibody (bs-2429R-AF647)
Dilution: 3μg /10^6 cells;
Isotype Control Antibody (orange line): Rabbit IgG-AF647.
Protocol
The cells were fixed with 4% PFA (10min at room temperature)and then were incubated in 5%BSA to block non-specific protein-protein interactions for 30 min at room temperature .Cells stained with Primary Antibody for 30 min at room temperature. Acquisition of 20,000 events was performed.

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