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ASM/Acid sphingomyelinase酸性神经鞘磷脂酶抗体，抗体质量可靠，订购
ASM/Acid sphingomyelinase酸性神经鞘磷脂酶抗体请联系在线客服或者销售人员。
抗体参数如下>>>>
中文名称：
酸性神经鞘磷脂酶抗体英文名称：
Anti-ASM/Acid sphingomyelinase货号：bs-6318R
抗体来源：兔
克隆类型：多克隆
蛋白分子量：predicted molecular weight: 64kDa
纯化方法：affinity purified by Protein A
交叉反应：hu, mo, rat, pig, dog, cow, Rb
测试应用：ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=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.
产品背景介绍：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.Subunit : Monomer.Subcellular Location : Lysosome.Similarity : Belongs to the acid sphingomyelinase family.Contains 1 saposin B-type domain.ASM酸性神经鞘磷脂酶是ASMase神经鞘磷脂酶Z重要的一个亚型,是细胞膜的重要组成成分。ASM在细胞凋亡、调节肿瘤细胞生长、参与Fas信号系统传递等方面均可发挥重要作用。
