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）

英文名称    BSCL2    

中文名称    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）    

别    名    Bernardinelli Seip congenital lipodystrophy 2; Bernardinelli Seip congenital lipodystrophy type 2 protein; Bernardinelli-Seip congenital lipodystrophy type 2 protein; BSCL 2; BSCL2; BSCL2_HUMAN; GNG3LG; HMN 5; HMN5; MGC4694; Seipin; Spastic paraplegia 17 (autosomal dominant); Spastic paraplegia 17 (Silver syndrome); Spastic paraplegia 17; Spastic paraplegia with amyotrophy of hands and feet (Silver syndrome); Spastic paraplegia with amyotrophy of hands and feet; SPG 17; SPG17.    

说 明 书    0.1ml  0.2ml      

研究领域    心血管  细胞生物  免疫学  神经生物学  细胞类型标志物      

抗体来源    Rabbit    

克隆类型    Polyclonal    

交叉反应    Human, Mouse, Rat, Dog, Cow, Horse, Rabbit,     

产品应用    WB=1:100-500 ELISA=1:500-1000 IP=1:20-100 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500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） （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.    

分 子 量    44kDa    

性    状    Lyophilized or Liquid    

浓    度    1mg/1ml    

免 疫 原    KLH conjugated synthetic peptide derived from human BSCL2/SPG17    

亚    型    IgG    

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    

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.    

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）产品介绍    background:

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.

Function:
Endoplasmic reticulum membrane; Multi-pass membrane protein.    

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）公司更多热销抗体产品如下：

hz-0293R-PE-hzy5.5 Rabbit Anti-rat IgG/PE-Cy5.5  PE-Cy5.5标记的兔抗大鼠IgG

hz-0293R-PE-hzy7 Rabbit Anti-rat IgG/PE-Cy7  PE-Cy7标记的兔抗大鼠IgG

hz-0346R-PE-hzY5 Rabbit Anti-rat IgM/PE-CY5  PE-CY5标记的兔抗大鼠IgM

hz-0346R-PE-hzy7 Rabbit Anti-rat IgM/PE-Cy7  PE-Cy7标记的兔抗大鼠IgM

hz-0346R-APhz Rabbit Anti-rat IgM/APC  APC标记的兔抗大鼠IgM

hz-0346R-AF350 Rabbit Anti-rat IgM/Alexa Fluor 350  Alexa Fluor 350标记的兔抗大鼠IgM

hz-0346R-AF488 Rabbit Anti-rat IgM/Alexa Fluor 488  Alexa Fluor 488标记的兔抗大鼠IgM

hz-0346R-AF555 Rabbit Anti-rat IgM/Alexa Fluor 555  Alexa Fluor 555标记的兔抗大鼠IgM

hz-0346R-AF647 Rabbit Anti-rat IgM/Alexa Fluor 647  Alexa Fluor 647标记的兔抗大鼠IgM

hz-0346R-PE-hzy5.5 Rabbit Anti-rat IgM/PE-Cy5.5  PE-Cy5.5标记的兔抗大鼠IgM

hz-0346R-RBIThz Rabbit Anti-rat IgM/RBITC  罗丹明标记的兔抗大鼠IgM

hz-0346R-FIThz Rabbit Anti-rat IgM/FITC  FITC标记的兔抗大鼠IgM

hz-0346R-AP Rabbit Anti-rat IgM/AP  碱性磷酸酶（AP）标记的兔抗大鼠IgM

hz-0346R-Gold Rabbit Anti-rat IgM/Gold  胶体金标记的兔抗大鼠IgM

hz-0346R-HRP Rabbit Anti-rat IgM/HRP  辣根过氧化物酶标记的兔抗大鼠IgM

hz-0346R-Bio Rabbit Anti-rat IgM/Bio  生物素标记的兔抗大鼠IgM

hz-0346R-hzy3 Rabbit Anti-rat IgM/Cy3  Cy3标记的兔抗大鼠IgM

hz-0346R-hzy5 Rabbit Anti-rat IgM/Cy5  Cy5标记的兔抗大鼠IgM

hz-0346R-hzy5.5 Rabbit Anti-rat IgM/Cy5.5  Cy5.5标记的兔抗大鼠IgM

hz-0346R-hzy7 Rabbit Anti-rat IgM/Cy7  Cy7标记的兔抗大鼠IgM

hz-0346R-PE Rabbit Anti-rat IgM/PE  PE标记的兔抗大鼠IgM

hz-0346R-PE-hzy3 Rabbit Anti-rat IgM/PE-Cy3  PE-Cy3标记的兔抗大鼠IgM

hz-0309R-Bio Rabbit Anti-pig IgG/Bio  生物素标记的兔抗猪IgG

hz-0309R-AF555 Rabbit Anti-pig IgG/Alexa Fluor 555  Alexa Fluor 555标记的兔抗猪IgG

hz-0309R-AP Rabbit Anti-pig IgG/AP  碱性磷酸酶（AP）标记的兔抗猪IgG

hz-0309R-HRP Rabbit Anti-pig IgG/HRP  辣根过氧化物酶标记的兔抗猪IgG