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產(chǎn)品詳情
  • 產(chǎn)品名稱:Anti-Acid sphingomyelinase抗體

  • 產(chǎn)品型號(hào):Anti-Acid sphingomyelinase
  • 產(chǎn)品廠商:KALANG
  • 產(chǎn)品文檔:
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Anti-Acid sphingomyelinase抗體產(chǎn)品質(zhì)量穩(wěn)定,實(shí)驗(yàn)效果明顯,貨期快,價(jià)格優(yōu)惠,歡迎垂詢訂購(gòu)!我公司長(zhǎng)期供應(yīng)**組化抗體、WB抗體、**組化試劑盒和抗體試驗(yàn)所需全部相關(guān)試劑、熒光標(biāo)記抗體、單克隆抗體、多克隆抗體、各種標(biāo)記的二抗IgG/IgM/IgD/IgA等科研實(shí)驗(yàn)抗體。Anti-Acid sphingomyelinase抗體用于**組化實(shí)驗(yàn),WB實(shí)驗(yàn),相應(yīng)的標(biāo)記抗體有HRP標(biāo)記抗體,FITC標(biāo)記,BIO等。
詳情介紹:
Rabbit  Anti-Acid sphingomyelinase
Cat. Number:
Anti-Acid sphingomyelinase抗體KL-6318R
Quantity size:
0.2ml
Concentration:
1mg/ml   Buffer = 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
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:Anti-Acid sphingomyelinase抗體 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 Anti-Acid sphingomyelinase抗體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.
Also known as:
Acid sphingomyelinase; Anti-Acid sphingomyelinase抗體ASM; ASM_HUMAN; aSMase; NPD; Smpd1; Sphingomyelin phosphodiesterase 1 acid lysosomal; Sphingomyelin phosphodiesterase.
Specificity:
Rabbit Polyclonal IgG, affinity purified by Protein A.
Reacts with: Human, Mouse, Rat, Dog, Pig, Cow, Rabbit, .
Immunogen: KLH conjugated synthetic peptide derived from human Acid sphingomyelinase.
Predicted Molecular Weight: 64kDa.
Storage:
Shipped at 4℃, Store at -20℃ (Avoid repeated freeze/thaw cycles).
Application:
WB=1:100-500  ELISA=1:500-1000  IHC-P=1:100-500  IHC-F=1:100-500  ICC=1:100-500  IF=1:100-500   
Not yet tested in other applications. 
Optimal working dilutionsAnti-Acid sphingomyelinase抗體 must be determined by the end user.
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kl262Mu01 轉(zhuǎn)鐵蛋白受體2(TFR2)多克隆抗體 Polyclonal Antibody to Transferrin Receptor 2 (TFR2)
kl262Ra01 轉(zhuǎn)鐵蛋白受體2(TFR2)多克隆抗體 Polyclonal Antibody to Transferrin Receptor 2 (TFR2)
kl263Bo01 線粒體肌酸激酶1A(CKMT1A)多克隆抗體 Polyclonal Antibody to Creatine Kinase, Mitochondrial 1A (CKMT1A)
kl263Ga01 線粒體肌酸激酶1A(CKMT1A)多克隆抗體 Polyclonal Antibody to Creatine Kinase, Mitochondrial 1A (CKMT1A)
kl263Hu01 線粒體肌酸激酶1A(CKMT1A)多克隆抗體 Polyclonal Antibody to Creatine Kinase, Mitochondrial 1A (CKMT1A)
kl263Mu01 線粒體肌酸激酶1A(CKMT1A)多克隆抗體 Polyclonal Antibody to Creatine Kinase, Mitochondrial 1A (CKMT1A)
kl263Po01 線粒體肌酸激酶1A(CKMT1A)多克隆抗體 Polyclonal Antibody to Creatine Kinase, Mitochondrial 1A (CKMT1A)
kl266Hu01 熱休克蛋白47(HSP47)多克隆抗體 Polyclonal Antibody to Heat Shock Protein 47 (HSP47)
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kl267Hu01 組織蛋白酶K(CTSK)多克隆抗體 Polyclonal Antibody to Cathepsin K (CTSK)
kl267Mu01 組織蛋白酶K(CTSK)多克隆抗體 Polyclonal Antibody to Cathepsin K (CTSK)
kl267Po01 組織蛋白酶K(CTSK)多克隆抗體 Polyclonal Antibody to Cathepsin K (CTSK)
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