本公司经销Dysferlin，Dysferlin蛋白抗体，克隆类型为polyclonal，宿主来源是Rabbit，DysferlinDysferlin蛋白抗体可应用于WB、elisa、IP、IF、IHC等实验，欢迎垂询订购！

货号：BY-2429R
英文名称：Anti-Dysferlin
中文名称：Dysferlin蛋白抗体
其他名称：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.
抗体来源：Rabbit
克隆类型：polyclonal
蛋白分子量：predicted molecular weight: 231kDa
纯化方法：affinity purified by Protein A
交叉反应：hu, mo, rat, Gpig, hrs, cow, Rb, dog
产品介绍：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].Subcellular Location : Cell membrane.Tissue Specificity : Expressed in skeletal muscle, myoblast, myotube ａnd in the syncytiotrophoblast （STB） of the placenta （at protein level）. Highly expressed in skeletal muscle. Also found in heart, brain, spleen, intestine, placenta ａnd at lower levels in liver, lung, kidney ａnd pancreas.DISEASE : Defects in DYSF are the cause of limb-girdle muscular dystrophy type 2B （LGMD2B） . LGMD2B is an autosomal recessive degenerative myopathy characterized by weakness ａnd atrophy starting in the proximal pelvifemoral muscles, with onset in the late teens or later, massive elevation of serum creatine kinase levels ａnd slow progression. Scapular muscle involvement is minor ａnd not present at onset. Upper limb girdle involvement follows some years after the onset in lower limbs.Dysferlin是与浆膜内环境有关的一种跨膜蛋白，与肌膜损伤的修复有关.产品图片Antigen: bs-2429P 0.2ug/100ul Primary: Antiserum, 1:500, 1:1000, 1:2000, 1:4000, 1:8000, 1:16000, 1:32000; Secondary: HRP conjugated Goat-Anti-Rabbit IgG（bs-0295G-HRP） at 1: 5000; TMB（C-0024） staining; Read the data in MicroplateReader by 450nm.