先天性脂肪代谢障碍蛋白2抗体（常染色体显性遗传痉挛性截瘫17）Anti-BSCL2/SPG17的产品详细说明：

－－产品介绍：Defects in BSCL2 are the cause of congenital generalized lipodystrophy type 2 （CGL2） . Congenital generalized lipodystrophy is an autosomal recessive disorder characterized by a near absence of adipose tissue, extreme insulin resistance, hypertriglyceridemia, hepatic steatosis ａnd early onset of diabetes.Defects in BSCL2 are the cause of spastic paraplegia type 17 （SPG17） ; also known as Silver spastic paraplegia syndrome. Spastic paraplegia is a neurodegenerative disorder characterized by a slow, gradual, progressive weakness ａnd spasticity of the lower limbs. SPG17 is characterized by prominent amyotrophy of the hand muscles, the presence of mild to severe pyramidal tract signs, ａnd spastic paraplegia. SPG17 is a motor neuron disease overlapping with distal spinal muscular atrophy type 5.Defects in BSCL2 are a cause of distal hereditary motor neuropathy type 5 （HMN5）; also known aS distal hereditary motor neuropathy type V （DSMAV）. HMN5 is an autosomal dominant disorder characterized by degeneration of motor nerve fibers, predominantly in limb distal regions.Tissue Specificity : Highest expression in brain ａnd testis.Similarity : Belongs to the seipin family.

（1mg/1ml先天性脂肪代谢障碍蛋白2抗体（常染色体显性遗传痉挛性截瘫17）Anti-BSCL2/SPG17，的来源为Rabbit，其相关产品：免疫组化常用试剂免疫印迹常用试剂各种标记二抗）

－－先天性脂肪代谢障碍蛋白2抗体（常染色体显性遗传痉挛性截瘫17）的参数标准：
浓度：1mg/1ml
交叉反应：hu, mo, rat, cow, dog, hrs, Rb
产品类型：一抗
研究领域：心血管神经生物学细胞类型标志物
蛋白分子量：predicted molecular weight: 44kDa
性状：Lyophilized or Liquid
免疫原：KLH conjugated synthetic peptide derived from human BSCL2/SPG17
亚型：polyclonal
纯化方法：affinity purified by Protein A
储存液：Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4
产品应用：WB=1:100-500ELISA=1:500-1000IP=1:20-100IHC-P=1:100-500IHC-F=1:100-500IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
保存条件：Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month ａnd for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.

上海田源专业销售先天性脂肪代谢障碍蛋白2抗体（常染色体显性遗传痉挛性截瘫17）抗体产品，详细抗体目录请访问：http://www.app17.com/c68810/products/t87312.html