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- Bioss
- bs-6318R-BF488
- 2025年09月30日
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100ul
| 产品编号 | bs-6318R-BF488 |
| 英文名称 | Acid sphingomyelinase Rabbit pAb, BF488 conjugated |
| 中文名称 | BF488标记的酸性神经鞘磷脂酶抗体 |
| 英文别名 | Acid sphingomyelinase; ASM; ASM_HUMAN; aSMase; NPD; Smpd1; Sphingomyelin phosphodiesterase 1 acid lysosomal; Sphingomyelin phosphodiesterase. |
| 产品应用 | Flow-Cyt=2ug/Test, IF=1:100-500 Not yet tested in other applications. |
| 抗体来源 | Rabbit |
| 免疫原 | KLH conjugated synthetic peptide derived from human Acid sphingomyelinase |
| 亚型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 克隆类型 | Polyclonal |
| 浓度 | 1mg/ml |
| 储存液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 研究领域 | Cell Biology > Apoptosis > Intracellular > Associated Proteins Neuroscience > Neurology process > Neurodegenerative disease Signal Transduction > Metabolism > Lipid metabolism |
| 亚基 | Monomer. |
| 亚细胞定位 | Lysosome. |
| 相似性 | Belongs to the acid sphingomyelinase family. Contains 1 saposin B-type domain. |
| 功能 | 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. |
| 保存条件 | Shipped at 4℃. Store at -20°C for one year. Avoid repeated freeze/thaw cycles. |
| 注意事项 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| 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.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. |
| 应用 | 推荐稀释比例 |
| {Flow-Cyt} | {2ug/Test} |
| {IF} | {1:100-500} |
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Acid sphingomyelinase Rabbit pAb, BF488 conjugated(bs-6318R-BF488)-100ul
￥2980
