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化驗
>99% (TLC)
形狀
powder
包裝
pkg of 1 × 50 μg (with stopper and crimp cap (810203P-50ug))
製造商／商標名
Avanti Research™ - A Croda Brand 810203P
運輸包裝
dry ice
儲存溫度
−20°C
一般說明
Lyso-sphingomyelin is a lysospingolipid used as a diagnostic biomarker. It allows a clear distinction between Niemann-Pick C disease (NP-C) and acid sphingomyelinase deficiency (ASMD).
應用
NBD Lyso SM or omega(7-nitro-2-1,3-benzoxadiazol-4-yl)-D-erythro-sphingosine-1-phosphocholine is suitable to determine the hydrolysis of sphingomyelin (SM) by RSp1609: acid sphingomyelinase-like phosphodiesterase (RsASML) protein.
包裝
2 mL Amber Serum Vial with Stopper and Crimp Cap (810203P-50ug)
法律資訊
Avanti Research is a trademark of Avanti Polar Lipids, LLC
儲存類別代碼
11 - Combustible Solids
水污染物質分類（WGK）
WGK 3
閃點（°F）
Not applicable
閃點（°C）
Not applicable
Federica Deodato et al.
Clinica chimica acta; international journal of clinical chemistry, 486, 387-394 (2018-08-29)
Although representing two distinct disease entities, Niemann-Pick disease type C (NP-C) disease and acid sphingomyelinase deficiency (ASMD) share several phenotypic features. The lack of biomarkers was responsible in the past of diagnostic delay. Recently, plasma oxysterols, cholestan-3β,5α,6β-triol (Triol) and 7-ketocholesterol
The impact of biomarkers analysis in the diagnosis of Niemann-Pick C disease and acid sphingomyelinase deficiency
Deodato F, et al.
Clinica Chimica Acta; International Journal of Clinical Chemistry, 486, 387-394 (2018)
Michael V Airola et al.
PloS one, 9(8), e105830-e105830 (2014-08-22)
Acid sphingomyelinase (aSMase) is a human enzyme that catalyzes the hydrolysis of sphingomyelin to generate the bioactive lipid ceramide and phosphocholine. ASMase deficiency is the underlying cause of the genetic diseases Niemann-Pick Type A and B and has been implicated
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