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42. Wolman disease/cholesteryl ester storage disease: Efficacy of plant-produced human lysosomal acid lipase in mice

✍ Scribed by Hong Du; Terri Cameron; Stephen Garger; Gregory Pogue; Lee Hamm; Earl White; Kathleen Hanley; Gregory Grabowski


Book ID
116988476
Publisher
Elsevier Science
Year
2009
Tongue
English
Weight
43 KB
Volume
96
Category
Article
ISSN
1096-7192

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Trinitrophenylaminolauric acid (TNPAL) was linked to glycerol or cholesterol and the resulting yellow compounds were used as substrates for several lipases and cholesteryl esterase in cells from normal individuals and patients with Wolman's or cholesteryl ester storage diseases. Normal cells (lympho