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Extended use of a selective inhibitor of acid lipase for the diagnosis of Wolman disease and cholesteryl ester storage disease

✍ Scribed by Civallero, G.; De Mari, J.; Bittar, C.; Burin, M.; Giugliani, R.


Book ID
122196781
Publisher
Elsevier Science
Year
2014
Tongue
English
Weight
250 KB
Volume
539
Category
Article
ISSN
0378-1119

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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