- 中文名称
Acid sphingomyelinase
- 英文名字
- Acid sphingomyelinase
- 供应商
- Nordic BioSite
- 产品货号
- NDC-BT-OBDZ4H-50
- 产品报价
- ￥询价/50ul
- 产品说明书
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- 购买方式
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- 产品新闻
- 背景资料
- 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.nInvolvement 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
- 应用类型
- FC
- 免疫原
- KLH conjugated synthetic peptide derived from human Acid sphingomyelinase:201-300/629
- 来源宿主
- rabbit
- 反应性
- human,mouse,rat,dog/canine,pig/porcine,cow/bovine,rabbit
- 保存建议
- Store at 4
- 其他
- Nordic BioSite(北欧生物) 总部位于瑞典，成立于1997年，旨在分销研究和诊断领域的高质量和创新产品。 Nordic BioSite 被公认为整个北欧生物医学研究和诊断产品领域的领导者。他们拥有超过570万种的产品组合，代理了遍布欧洲和美国的顶级生物试剂制造商，是欧洲知名的一站式生物试剂代理商。除了作为分销商之外，Nordic BioSite 还拥有不断增长的自产产品线——BioSite mAb、BioSite ELISA、BioSite Flow、Optibodies和 BioSite Protein, 目前艾美捷代理的是Nordic BioSite自产产品线(共26W+产品)。Nordic BioSite的产品类型主要是抗体，蛋白和试剂盒，产品适用范围涵盖15个不同的物种(人，牛，犬，鲶鱼，鸡，海豚，马，猫，豚鼠，小鼠，绵羊，兔子，大鼠，猪和火鸡)，可为您提供一站式的生命科学研究解决方案~
- 注意
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该页面的中文产品信息的翻译，仅供参考。准确的产品信息请以厂家的英文说明书为准。下单前，请浏览说明书确认。
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