Participants in this research study have an inherited condition that results in an elevated level of cholestanol (a break-down product of cholesterol) in the blood and tissues and a very reduced amount of a bile acid called chenodeoxycholic in the bile. The purpose of the study is to: 1) determine if feeding a diet that contains no cholestanol and no cholesterol will lower the amount of cholestanol in the blood 2) determine if taking simvastatin (Zocor), a drug that lowers the level of cholesterol in the blood and taking chenodeoxycholic acid will lower the level of cholestanol in the blood and reduce the size of the patient's xanthoma and make them feel better, 3) determine if and how much cholesterol and cholestanol are removed from the Achilles tendon after two years on medication and 4) use cells from a skin biopsy to study the genetics of CTX (cerebrotendinous xanthomatosis).

Agency
National Institute of Health (NIH)
Institute
National Center for Research Resources (NCRR)
Type
General Clinical Research Centers Program (M01)
Project #
2M01RR000334-34
Application #
6411367
Study Section
Special Emphasis Panel (ZRR1)
Project Start
1976-12-01
Project End
2004-11-30
Budget Start
Budget End
Support Year
34
Fiscal Year
2000
Total Cost
Indirect Cost
Name
Oregon Health and Science University
Department
Type
DUNS #
009584210
City
Portland
State
OR
Country
United States
Zip Code
97239
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