FITC标记的酸性神经鞘磷脂酶抗体
产品名称： FITC标记的酸性神经鞘磷脂酶抗体
英文名称： Anti-Acid sphingomyelinase/FITC
产品编号： HZ-
产品价格： null
产品产地： 中国/上海
品牌商标： HZbscience
更新时间： 2023-08-17T10:24:20
使用范围： ICC=1:50-200 IF=1:50-200
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Rabbit Anti-Acid sphingomyelinase/FITC Conjugated antibody
FITC标记的酸性神经鞘磷脂酶抗体
| 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. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 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 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 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. Subunit: Monomer. Subcellular Location: Lysosome. DISEASE: Defects in SMPD1 are the cause of Niemann-Pick disease type A (NPDA) [MIM:257200]; 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. Defects in SMPD1 are the cause of Niemann-Pick disease type B (NPDB) [MIM:607616]; also known as Niemann-Pick disease visceral form. It is a late-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. Clinical signs involve only visceral organs. The most constant sign is hepatosplenomegaly which can be associated with pulmonary symptoms. Patients remain free of neurologic manifestations. However, 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. In Niemann-Pick disease type B, onset of the first symptoms occurs in early childhood and patients can survive into adulthood. Similarity: Belongs to the acid sphingomyelinase family. Contains 1 saposin B-type domain. Database links: Entrez Gene: 505097 Cow Entrez Gene: 485334 Dog Entrez Gene: 100720041 Guinea pig Entrez Gene: 6609 Human Entrez Gene: 20597 Mouse Entrez Gene: 100353898 Rabbit Entrez Gene: 308909 Rat Omim: 607608 Human SwissProt: Q0VD19 Cow SwissProt: P17405 Human SwissProt: Q04519 Mouse Unigene: 498173 Human Unigene: 4628 Mouse Unigene: 485064 Mouse Unigene: 18277 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. ASM酸性神经鞘磷脂酶是ASMase神经鞘磷脂酶最重要的一个亚型,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 |
将鞘磷脂转化为神经酰胺。磷脂酶C对1,2-二酰基甘油磷酸胆碱和1,2-二酰基甘油***具有磷脂酶C活性。异构体2和异构体3已经失去催化活性。
参与疾病：SMPD1的缺陷是尼曼匹克病A型（NPDA）的原因；也被称为尼曼匹克病经典婴儿型。这是一种早发性溶酶体贮积症，由于未能将鞘磷脂水解成神经酰胺。它导致鞘磷脂和其他代谢相关脂质在网状内皮和其他细胞类型的积累，在整个身体，导致细胞死亡。A型尼曼-匹克病是一种主要的神经退行性疾病，其特征是在生命的第一年内发病，智力低下，消化障碍，发育不全，主要肝脾肿大，以及严重的神经症状。严重的神经系统疾病和肺部感染导致早死，通常在四岁左右。临床特征是可变的。A型（基本神经内脏）和B型（纯内脏）型尼曼匹克病之间存在表型连续性，中间型包含A和B两种临床特征相结合的变异群。