產品詳情
簡單介紹：
Anti-Acid sphingomyelinase抗體產品質量穩定，實驗效果明顯，貨期快，價格優惠，歡迎垂詢訂購！我公司長期供應**組化抗體、WB抗體、**組化試劑盒和抗體試驗所需全部相關試劑、熒光標記抗體、單克隆抗體、多克隆抗體、各種標記的二抗IgG/IgM/IgD/IgA等科研實驗抗體。Anti-Acid sphingomyelinase抗體用于**組化實驗,WB實驗,相應的標記抗體有HRP標記抗體,FITC標記,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.
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:
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Rabbit Polyclonal IgG, affinity purified by Protein A.
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Reacts with: Human, Mouse, Rat, Dog, Pig, Cow, Rabbit, .
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Immunogen: KLH conjugated synthetic peptide derived from human Acid sphingomyelinase.
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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.
Not yet tested in other applications.
Optimal working dilutionsAnti-Acid sphingomyelinase抗體 must be determined by the end user.
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