| 克隆类型 ： |
Rabbit Polyclonal IgG |
|
免 疫 原 ： |
Peptide derived from human Dysferlin. |
|
纯化方式 ： |
Immunogen affinity purified |
|
缓 冲 液 ： |
PBS, pH 7.4(1%BSA and 0.1% Sodium azide) |
|
产品描述 ： |
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. |
| 适用物种 ： |
Hu, Mo, Rat, Dog, Pig, Cow, Hor, Guinea Pig |
| 应用说明 ： |
WB: 1:200-1:1000 E: 1:500-1:1000 IHC: 1:200-1:1000 IF: 1:100-1:500 |
| 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. |
| 保存条件 ： |
Store at -20°C for one year. Avoid repeated freeze-thaw cycles. At 4°C for 2-3 weeks after reconstituted. |
| 标 记 物 ： |
PLLABS公司可供应生物素、辣根过氧化物酶、荧光素标记抗体 |
|
产品说明 ： |
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