Selective sterol accumulation in ABCG5/ABCG8-deficient mice

L Yu, K Von Bergmann, D Lutjohann, HH Hobbs… - Journal of lipid …, 2004 - ASBMB
The ATP binding cassette (ABC) transporters ABCG5 and ABCG8 limit intestinal absorption
and promote biliary secretion of neutral sterols. Mutations in either gene cause
sitosterolemia, a rare recessive disease in which plasma and tissue levels of several neutral
sterols are increased to varying degrees. To determine why patients with sitosterolemia
preferentially accumulate noncholesterol sterols, levels of cholesterol and the major plant
sterols were compared in plasma, liver, bile, and brain of wild-type and ABCG5/ABCG8 …