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A practical fluorometric assay method to measure lysosomal acid lipase activity in dried blood spots for the screening of cholesteryl ester storage disease.

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Presentation on theme: "A practical fluorometric assay method to measure lysosomal acid lipase activity in dried blood spots for the screening of cholesteryl ester storage disease."— Presentation transcript:

1 A practical fluorometric assay method to measure lysosomal acid lipase activity in dried blood spots for the screening of cholesteryl ester storage disease and Wolman disease  Takenori Dairaku, Takeo Iwamoto, Minami Nishimura, Masahiro Endo, Toya Ohashi, Yoshikatu Eto  Molecular Genetics and Metabolism   Volume 111, Issue 2, Pages (February 2014) DOI: /j.ymgme Copyright © 2013 Elsevier Inc. Terms and Conditions

2 Fig. 1 Excitation spectra (emission wavelength=450nm) of 20nM 4-methylumbelliferone at pH values 4.1 (a), 7.1 (b), 8.4 (c) and 10.1 (d). Molecular Genetics and Metabolism   , DOI: ( /j.ymgme ) Copyright © 2013 Elsevier Inc. Terms and Conditions

3 Fig. 2 Emission spectra at excitation wavelength=365nm (A) and emission spectra at excitation wavelength=320nm (B) of 20nM 4-methylumbelliferone at pH values 4.1 (a), 7.1 (b), 8.4 (c) and 10.1 (d). Molecular Genetics and Metabolism   , DOI: ( /j.ymgme ) Copyright © 2013 Elsevier Inc. Terms and Conditions

4 Fig. 3 Comparison of the LAL activity of 51 normal, 7 obligate carriers and 7 CESD samples. Molecular Genetics and Metabolism   , DOI: ( /j.ymgme ) Copyright © 2013 Elsevier Inc. Terms and Conditions

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