酸性神经鞘磷脂酶抗体Anti-ASM/Acid sphingomyelinase的产品详细说明：

－－产品介绍：Converts sphingomyelin to ceramide. Also has phospholipase C activities toward 1,2-diacylglycerolphosphocholine ａnd 1,2-diacylglycerolphosphoglycerol. Isoform 2 ａnd isoform 3 have lost catalytic activity.Involvement in disease: Defects in SMPD1 are the cause of Niemann-Pick disease type A （NPDA） ; also known as Niemann-Pick disease classical infantile form. It is an early-onset lysosomal storage disorder caused by failure to hydrolyze sphingomyelin to ceramide. It results in the accumulation of sphingomyelin ａnd other metabolically related lipids in reticuloendothelial ａnd other cell types throughout the body, leading to cell death. Niemann-Pick disease type A is a primarily neurodegenerative disorder characterized by onset within the first year of life, mental retardation, digestive disorders, failure to thrive, major hepatosplenomegaly, ａnd severe neurologic symptoms. The severe neurological disorders ａnd pulmonary infections lead to an early death, often around the age of four. Clinical features are variable. A phenotypic continuum exists between type A （basic neurovisceral） ａnd type B （purely visceral） forms of Niemann-Pick disease, ａnd the intermediate types encompass a cluster of variants combining clinical features of both types A ａnd B.

（1mg/1ml酸性神经鞘磷脂酶抗体Anti-ASM/Acid sphingomyelinase，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）

－－酸性神经鞘磷脂酶抗体的参数标准：
浓度：1mg/1ml
交叉反应：hu, mo, rat, Sheep, Cow, Dog
产品类型：一抗
研究领域：细胞生物神经生物学信号转导细胞凋亡
蛋白分子量：predicted molecular weight: 70kDa
性状：Lyophilized or Liquid
免疫原：KLH conjugated synthetic peptide derived from hu ASM/Acid sphingomyelinase
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.

上海田源专业销售酸性神经鞘磷脂酶抗体抗体产品，详细抗体目录请访问：http://www.app17.com/c68810/products/t87312.html