Sphingomyelin phosphodiesterase 1 (SMPD1) , also known as ASM ( acid sphingomyelinase ), is a member of the acid sphingomyelinase family of enzymes. Three isoforms have been identified, isoform 1 is 631 amino acids (aa) in length as the pro form, while Isoform 2 and isoform 3 have lost catalytic activity. The active SMPD1 isoform 1 contains one saposin B-type domain that likely interacts with sphingomyelin, and a catalytic region. Human SMPD1 is 86% aa identical to mouse SMPD1. SMPD1 is a monomeric lysosomal enzyme that converts sphingomyelin (a plasma membrane lipid ) into ceramide through the removal of phosphorylcholine. This generates second messenger components that participate in signal transduction. Defects in SMPD1 are the cause of Niemann-Pick disease type A (NPA) and type B (NPB), also known as Niemann-Pick disease classical infantile form and Niemann-Pick disease visceral form. Niemann-Pick disease is a clinically and genetically heterogeneous recessive disorder. NPB has little if any neurologic involvement and patients may survive into adulthood.
规格 | 价格/CNY | 货期 | 数量 | |
---|---|---|---|---|
50 μg | ¥ 3,820 | 现货 | ||
100 μg | ¥ 6,530 | 5日内发货 | ||
200 μg | ¥ 11,130 | 5日内发货 | ||
500 μg | ¥ 22,510 | 5日内发货 |
生物活性 | Measured by its ability to cleave 2-N-Hexadecanoylamino-4-nitrophenylphosphorylcholine(HNPPC). The specific activity is > 1,000 pmoles/min/μg. |
产品描述 | Sphingomyelin phosphodiesterase 1 (SMPD1) , also known as ASM ( acid sphingomyelinase ), is a member of the acid sphingomyelinase family of enzymes. Three isoforms have been identified, isoform 1 is 631 amino acids (aa) in length as the pro form, while Isoform 2 and isoform 3 have lost catalytic activity. The active SMPD1 isoform 1 contains one saposin B-type domain that likely interacts with sphingomyelin, and a catalytic region. Human SMPD1 is 86% aa identical to mouse SMPD1. SMPD1 is a monomeric lysosomal enzyme that converts sphingomyelin (a plasma membrane lipid ) into ceramide through the removal of phosphorylcholine. This generates second messenger components that participate in signal transduction. Defects in SMPD1 are the cause of Niemann-Pick disease type A (NPA) and type B (NPB), also known as Niemann-Pick disease classical infantile form and Niemann-Pick disease visceral form. Niemann-Pick disease is a clinically and genetically heterogeneous recessive disorder. NPB has little if any neurologic involvement and patients may survive into adulthood. |
种属 | Mouse |
表达系统 | Baculovirus-Insect Cells |
标签 | His |
蛋白编号 | Q04519 |
别名 | aSMase, ASM, Zn-SMase, A-SMase, sphingomyelin phosphodiesterase 1 |
蛋白构建 | A DNA sequence encoding the mouse SMPD1 (Q04519) (Met 1-Leu 626) was expressed,with a C-terminal polyhistidine tag. |
蛋白纯度 |
> 85 % as determined by SDS-PAGE
|
分子量 | Approxiamtely 66.3 kDa |
内毒素 | < 1.0 EU per μg of the protein as determined by the LAL method. |
缓冲液 | Supplied as sterile 20mM Tris, 500mM NaCl, 10% glycerol, pH 8.0, 0. 1% Tween20Please contact us for any concerns or special requirements. Please refer to the specific buffer information in the hard copy of CoA. |
复溶方法 | A hardcopy of COA with reconstitution instruction is sent along with the products. Please refer to it for detailed information. |
存储 |
Samples are stable for up to twelve months from date of receipt at -20℃ to -80℃. Store it under sterile conditions at -20℃ to -80℃. It is recommended that the protein be aliquoted for optimal storage. Avoid repeated freeze-thaw cycles. |
运输方式 |
Solution. It is shipped out with blue ice. |
研究背景 | Sphingomyelin phosphodiesterase 1 (SMPD1) , also known as ASM ( acid sphingomyelinase ), is a member of the acid sphingomyelinase family of enzymes. Three isoforms have been identified, isoform 1 is 631 amino acids (aa) in length as the pro form, while Isoform 2 and isoform 3 have lost catalytic activity. The active SMPD1 isoform 1 contains one saposin B-type domain that likely interacts with sphingomyelin, and a catalytic region. Human SMPD1 is 86% aa identical to mouse SMPD1. SMPD1 is a monomeric lysosomal enzyme that converts sphingomyelin (a plasma membrane lipid ) into ceramide through the removal of phosphorylcholine. This generates second messenger components that participate in signal transduction. Defects in SMPD1 are the cause of Niemann-Pick disease type A (NPA) and type B (NPB), also known as Niemann-Pick disease classical infantile form and Niemann-Pick disease visceral form. Niemann-Pick disease is a clinically and genetically heterogeneous recessive disorder. NPB has little if any neurologic involvement and patients may survive into adulthood. |
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Acid sphingomyelinase/SMPD1 Protein, Mouse, Recombinant (His) aSMase ASM Zn-SMase A-SMase sphingomyelin phosphodiesterase 1 recombinant recombinant-proteins proteins protein