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酸性神經(jīng)鞘磷脂酶抗體
  • 產(chǎn)品貨號：
    BN41938R
  • 中文名稱：
    酸性神經(jīng)鞘磷脂酶抗體
  • 英文名稱：
    Rabbit anti-Acid sphingomyelinase Polyclonal antibody
  • 品牌：
    Biorigin

  • 貨號

    產(chǎn)品規(guī)格

    售價

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  • BN41938R-50ul

    50ul

    ￥1486.00

    交叉反應(yīng)：Human,Mouse,Rat(predicted:Dog,Pig,Cow,Rabbit) 推薦應(yīng)用：WB,IHC-P,IHC-F,ICC,IF,Flow-Cyt,ELISA

  • BN41938R-100ul

    100ul

    ￥2360.00

    交叉反應(yīng)：Human,Mouse,Rat(predicted:Dog,Pig,Cow,Rabbit) 推薦應(yīng)用：WB,IHC-P,IHC-F,ICC,IF,Flow-Cyt,ELISA

  • BN41938R-200ul

    200ul

    ￥3490.00

    交叉反應(yīng)：Human,Mouse,Rat(predicted:Dog,Pig,Cow,Rabbit) 推薦應(yīng)用：WB,IHC-P,IHC-F,ICC,IF,Flow-Cyt,ELISA

產(chǎn)品描述

英文名稱Acid sphingomyelinase
中文名稱酸性神經(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(xì)胞生物  神經(jīng)生物學(xué)  信號轉(zhuǎn)導(dǎo)  細(xì)胞凋亡  
抗體來源Rabbit
克隆類型Polyclonal
交叉反應(yīng)Human, Mouse, Rat,  (predicted: Dog, Pig, Cow, Rabbit, )
產(chǎn)品應(yīng)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(fù)）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量64kDa
細(xì)胞定位細(xì)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.
PubMedPubMed
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: 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酸性神經(jīng)鞘磷脂酶是ASMase神經(jīng)鞘磷脂酶最重要的一個亞型,是細(xì)胞膜的重要組成成分。ASM在細(xì)胞凋亡、調(diào)節(jié)腫瘤細(xì)胞生長、參與Fas信號系統(tǒng)傳遞等方面均可發(fā)揮重要作用。





























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