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FITC标记的酸性神经鞘磷脂酶抗体
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价格：￥电议
更新日期：2026/8/7 18:14:45
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相关标签：FITC标记的酸性神经鞘磷脂酶抗体
详细内容
| 英文名称 | Anti-Acid sphingomyelinase/FITC |
| 中文名称 | FITC标记的酸性神经鞘磷脂酶抗体 |
| 别 名 | Acid sphingomyelinase; ASM; ASM_HUMAN; aSMase; NPD; Smpd1; Sphingomyelin phosphodiesterase 1 acid lysosomal; Sphingomyelin phosphodiesterase. |
| 说 明 书 | 100ul |
| 研究领域 | 细胞生物 神经生物学 信号转导 细胞凋亡 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Dog, Pig, Cow, Rabbit, |
| 产品应用 | ICC=1:50-200 IF=1:50-200 not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 64kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human Acid sphingomyelinase |
| 亚 型 | IgG |
| 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 |
| 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. |
| 产品介绍 | background: 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. Function: 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. |
FITC标记的酸性神经鞘磷脂酶抗体
