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Updated: Jul 14, 2026

A Familial Hypercholesterolemia Human Liver Chimeric Mouse Model Using Induced Pluripotent Stem Cell-derived Hepatocytes
Published on: September 15, 2018
Stearoyl-CoA desaturase deficiency, hypercholesterolemia, cholestasis, and diabetes
Alan D Attie1, Matthew T Flowers, Jessica B Flowers
1Department of Biochemistry, University of Wisconsin, Madison, Wisconsin 53706-1544, USA. attie@biochem.wisc.edu
Abstract:
Previous studies showed that mice deficient in Scd1 had a reduced level of liver triglyceride and an improvement in insulin sensitivity. We studied Scd1(-/-) mice on a very low-fat, high-carbohydrate lipogenic diet. The animals were almost entirely devoid of high-density lipoprotein (HDL). Nonetheless, they were hypercholesterolemic and had cholestasis. These changes were reversible with oil containing both mono- and polyunsaturated fat, but not entirely reversible with just triolein, suggesting that Scd1 deficiency increased the requirement for polyunsaturated fat. We also found that the Scd1(-/-) mice on a normal chow diet had dramatically improved insulin sensitivity. However, leptin(ob/ob) Scd1(-/-) mice had worse diabetes than leptin(ob/ob) Scd1(wt/wt) mice.
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