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蛋白质/抗原/多肽 >> 重组蛋白
重组先天性脂肪代谢障碍蛋白2(常染色体显性遗传痉挛性截瘫17)
重组先天性脂肪代谢障碍蛋白2(常染色体显性遗传痉挛性截瘫17)图片
交货期: 1周
索取资料及报价
交货期:1周
产品别名:Recombinant Bscl2
Recombinant Berardinelli-Seip congenital lipodystrophy 2 (seipin) protein
产品介绍
基因名:

Bscl2


产品别名:

2900097C17Rik; AI046355; Gng3lg; Bscl2; Berardinelli-Seip congenital lipodystrophy 2 (seipin); Berardinelli-Seip congenital lipodystrophy 2 (seipin); seipin; Berardinelli-Seip congenital lipodystrophy 2 homolog (seipin); Bernardinelli-Seip congenital lipodystrophy 2 homolog; G protein gamma 3 linked; bernardinelli-Seip congenital lipodystrophy type 2 protein homolog; 先天性脂肪代谢障碍蛋白2(常染色体显性遗传痉挛性截瘫17);


背景信息:
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.

标签:His-tag   
分类:Recombinant  
类型:Protein   
偶联物:Unconjugated
内毒素水平:按批次,参阅瓶身标签
性状:Liquid
浓度:Batch dependent (Please refer to the vial label for the specific concentration.)
纯化类型:purified
内含物:不含防腐剂
应用:Positive Control;Immunogen;SDS-PAGE;WB.
储存:2-8°C不超过一个月,-80°C不超过12个月。避免反复冻融。
注意事项:仅供实验室使用。不适用于人类或动物的任何临床,治疗或诊断用途。不适合动物或人类食用。

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