Acid lipase cross-reacting material in Wolman disease and cholesterol ester storage disease

B. K. Burton, S. P. Reed

Research output: Contribution to journalArticlepeer-review

16 Scopus citations

Abstract

Material cross-reacting with antibodies to acid lipase was demonstrated in fibroblasts of three patients with Wolman disease and three with cholesterol ester storage disease. Quantitation of the immunologically cross-reacting material (CRM) by a single radial immunodiffusion method revealed normal levels in both mutant cell types. CRM specific activity toward triolein and cholesteryl oleate was reduced about 200-fold in the Wolman disease fibroblasts and 50- to 100-fold in the cholesterol ester storage disease cells when compared to normal.

Original languageEnglish (US)
Pages (from-to)203-208
Number of pages6
JournalAmerican Journal of Human Genetics
Volume33
Issue number2
StatePublished - Jan 1 1981

ASJC Scopus subject areas

  • Genetics
  • Genetics(clinical)

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