GERALD SALEN and ScoTT

Dr. Salen is a recipient of a Clinical Investigatorship Award from the Veterans Administration, Washington, D. C. His present address is Veterans Administration Hospital, East Orange, N. J. 07019. Received for publication 23 January 1973 and in revised form 27 June 1973. tanol outputs. In contrast, the administration of clofibrate lowered plasma cholesterol levels 50% and presumably reduced synthesis in the CTX subjects. Plasma cholesterol concentrations and fecal steroid excretion did not change significantly during this therapy. These findings indicate that the excessive tissue deposits of cholesterol and cholestanol that characterize CTX were associated with hyperactive neutral sterol synthesis. The demonstration of subnormal bile acid formation suggests that defective bile acid synthesis may predispose to the neutral sterol abnormalities.