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BN41938R-50ul
50ul
￥1486.00
交叉反應：Human,Mouse,Rat(predicted:Dog,Pig,Cow,Rabbit) 推薦應用：WB,IHC-P,IHC-F,ICC,IF,Flow-Cyt,ELISA
BN41938R-100ul
100ul
￥2360.00
交叉反應：Human,Mouse,Rat(predicted:Dog,Pig,Cow,Rabbit) 推薦應用：WB,IHC-P,IHC-F,ICC,IF,Flow-Cyt,ELISA
BN41938R-200ul
200ul
￥3490.00
交叉反應：Human,Mouse,Rat(predicted:Dog,Pig,Cow,Rabbit) 推薦應用：WB,IHC-P,IHC-F,ICC,IF,Flow-Cyt,ELISA
產(chǎn)品描述
| 英文名稱(chēng) | Acid sphingomyelinase |
| 中文名稱(chēng) | 酸性神經(jīng)鞘磷脂酶抗體 |
| 別 名 | Acid sphingomyelinase; ASM; ASM_HUMAN; aSMase; NPD; Smpd1; Sphingomyelin phosphodiesterase 1 acid lysosomal; Sphingomyelin phosphodiesterase. |
| 研究領(lǐng)域 | 細胞生物 神經(jīng)生物學(xué) 信號轉導 細胞凋亡 |
| 抗體來(lái)源 | Rabbit |
| 克隆類(lèi)型 | Polyclonal |
| 交叉反應 | Human, Mouse, Rat, (predicted: Dog, Pig, Cow, Rabbit, ) |
| 產(chǎn)品應用 | WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 Flow-Cyt=2ug/Test ICC=1:100-500 IF=1:100-500 （石蠟切片需做抗原修復） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 64kDa |
| 細胞定位 | 細胞漿 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human Acid sphingomyelinase:201-300/629 |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 儲 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存條件 | Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. |
| PubMed | PubMed |
| 產(chǎn)品介紹 | 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. SWISS: P17405 Gene ID: 6609 Database links: Entrez Gene: 100720041 Guinea pig Entrez Gene: 6609 Human Entrez Gene: 20597 Mouse Entrez Gene: 100353898 Rabbit Omim: 607608 Human 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酸性神經(jīng)鞘磷脂酶是ASMase神經(jīng)鞘磷脂酶最重要的一個(gè)亞型,是細胞膜的重要組成成分。ASM在細胞凋亡、調節腫瘤細胞生長(cháng)、參與Fas信號系統傳遞等方面均可發(fā)揮重要作用。 |
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