New spectrophotometric assays of acid lipase and their use in the diagnosis of wolman and cholesteryl ester storage diseases

Anne Nègre, Robert Salvayre, Arie Dagan, Carla Borrone, Shimon Gatt

Research output: Contribution to journalArticlepeer-review

16 Scopus citations

Abstract

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 (lymphoid cell lines or skin fibroblasts) showed two peaks of lipase or cholesteryl esterase activity at about pH 4.0 and 6.0 each. The activity of the most acidic enzyme, which hydrolyzed natural or synthetic triacylglycerols as well as cholesteryl esters, was considerably reduced in cells derived from patients with Wolman's or cholesteryl ester storage diseases. Simple spectrophotometric procedures were developed for using tri-TNPAL glycerol or TNPAL cholesterol to identify homozygotes of these two respective diseases.

Original languageEnglish
Pages (from-to)398-405
Number of pages8
JournalAnalytical Biochemistry
Volume145
Issue number2
DOIs
StatePublished - Mar 1985
Externally publishedYes

Keywords

  • Wolman's disease
  • acid cholesteryl esterase
  • acid lipase
  • cholesteryl ester storage disease
  • trinitrophenylaminolauryl cholesterol
  • trinitrophenylaminolauryl triglyceride

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