Saito Y
Second Department of Internal Medicine, School of Medicine, Chiba University, Japan.
Gerontology. 1991;37 Suppl 1:48-55. doi: 10.1159/000213297.
Abnormal lipoprotein metabolism and its cause were studied in 10 patients with Werner's syndrome. Seven of the 10 patients had hypercholesterolemia (greater than 250 mg dl-1). A significant positive correlation (p less than 0.01) was found between serum total cholesterol levels and LDL receptor activity. In monocyte-derived macrophages of patients with this syndrome, the uptake, lysosomal hydrolysis and re-esterification of free cholesterol are enhanced. This abnormal accumulation of cholesterol ester may cause accelerated conversion of macrophages to foam cells in Werner's syndrome.
对10例沃纳综合征患者的异常脂蛋白代谢及其病因进行了研究。10例患者中有7例患有高胆固醇血症(大于250mg/dl)。血清总胆固醇水平与低密度脂蛋白受体活性之间存在显著正相关(p < 0.01)。在该综合征患者的单核细胞衍生巨噬细胞中,游离胆固醇的摄取、溶酶体水解和再酯化均增强。胆固醇酯的这种异常蓄积可能导致沃纳综合征中巨噬细胞加速转化为泡沫细胞。