6-Hexadecanoylamino-4-methylumbelliferyl-phosphorylcholine
Purity: Free 6HMU < 0.1 %
Biochemical test:
Sphingomyelinase activity in fibroblasts and leukocytes from
Niemann-Pick A patients < 4.2 % and < 9 % of mean
normal activity, respectively.
Molecular Formula:
C31H51N2O7P MW: 595
Literature reference: A new fluorimetric enzyme assay for the diagnosis of Niemann-
Pick A/B, with specificity of natural sphingomyelinase substrate.
van Diggelen OP, Voznyi YV, Keulemans JLM, Schoonderwoerd K,
Ledvinova L, Mengel E, Zschiesche M, Santer R, Harzer K.
J Inher Metab Dis 2005; 28: 733-741