产品详情
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.
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