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

The American Journal of Human Genetics (Impact Factor: 10.99). 04/1981; 33(2):203-8.
Source: PubMed

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.

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