沃尔曼病和胆固醇酯贮积病中的酸性脂肪酶交叉反应物质。

Burton B K, Reed S P

Am J Hum Genet. 1981 Mar;33(2):203-8.

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.

在三名患有沃尔曼病和三名患有胆固醇酯贮积病的患者的成纤维细胞中，证实了与酸性脂肪酶抗体发生交叉反应的物质。通过单向放射免疫扩散法对免疫交叉反应物质（CRM）进行定量分析，结果显示两种突变细胞类型中的CRM水平均正常。与正常细胞相比，沃尔曼病成纤维细胞中针对三油酸甘油酯和油酸胆固醇酯的CRM比活性降低了约200倍，而胆固醇酯贮积病细胞中的CRM比活性降低了50至100倍。