| Abstract: | In view of the proposed membrane defect in Huntington's disease, cultured skin fibroblasts from healthy volunteers and patients with Huntington's disease were compared with respect to their ability to carry out de novo synthesis of cholesterol. At confluency, values for incorporation of 14C]acetate and 3H2O into cholesterol, and activities of HMG-CoA reductase (the rate-limiting enzyme in the cholesterol biosynthetic pathway), did not differ significantly in the Huntington's disease cells compared to the controls. Determinations of total cellular cholesterol gave similar ratios of cholesterol/protein and cholesterol/phospholipid in the Huntington's disease and control fibroblasts. The data suggest that the proposed generalized cell membrane abnormality in Huntington's disease cannot be attributed to a defect in the cholesterol biosynthetic pathway. |
