Biliary excretion of glycolipid in induced or inherited glucosylceramide lipidosis.

Metabolically inert L-[1-14C]glucosylceramide is stored predominantly in the liver after intravenous administration to mice. The half-time of this glycolipid analogue in the liver is 3.5 days and its clearance occurs predominantly via the bile. Within the limited number of Gaucher specimens available for examination very high levels of glucosylceramide were found in the bile of one patient and in the liver of two patients with biliary obstruction. The question of a possible relationship between biliary excretion of glycolipid and the pathogenesis of Gaucher's disease will require further studies.