The level of serum lipids, vitamin E and low density lipoprotein oxidation in Wilson's disease patients.
The aim of this study was to estimate the level of lipids and of the main serum antioxidant, alpha-tocopherol (vitamin E), and to evaluate the susceptibility of low density lipoprotein (LDL) to oxidation in Wilson's disease patients. It was assumed that enhanced LDL peroxidation caused by high copper levels could contribute to the injury of liver and other tissues. The group investigated comprised 45 individuals with Wilson's disease treated with penicillamine or zinc salts and a control group of 36 healthy individuals. Lipids were determined by enzymatic methods, alpha-tocopherol by high performance liquid chromatography, the susceptibility of LDL to oxidation in vitro by absorption changes at 234 nm during 5 h and end-products of LDL lipid oxidation as thiobarbituric acid reacting substances. In Wilson's disease patients total cholesterol, LDL cholesterol and alpha-tocopherol levels were significantly lower compared with the control group. No difference in LDL oxidation in vitro between the patients and the controls was stated.


CONCLUSIONS
enhanced susceptibility of isolated LDL for lipid peroxidation in vitro was not observed in Wilson's disease patients. One cannot exclude, however, that because of low alpha-tocopherol level lipid peroxidation in the tissues can play a role in the pathogenesis of tissue injury in this disease.
Rodo
M
M
Department of Genetics, 2nd Department of Neurology and Analytical Laboratory, Institute of Psychiatry and Neurology, ul. Sobieskiego 1/9, 02-957 Warsaw, Poland. Rodo@ipin.edu.pl
Czonkowska
A
A
Pulawska
M
M
Swiderska
M
M
Tarnacka
B
B
Wehr
H
H
D000328:Adult; D002784:Cholesterol / Q000097:blood; D003300:Copper / Q000097:blood; D006527:Hepatolenticular Degeneration / Q000097:blood* / Q000188:drug therapy; D006801:Humans; D015227:Lipid Peroxidation / Q000502:physiology; D008077:Lipoproteins, LDL / Q000097:blood*; D008875:Middle Aged; D010396:Penicillamine / Q000008:administration & dosage; D016896:Treatment Outcome; D014280:Triglycerides / Q000097:blood; D014810:Vitamin E / Q000097:blood*; D019287:Zinc Sulfate / Q000008:administration & dosage
D008077:Lipoproteins, LDL; D014280:Triglycerides; D014810:Vitamin E; D019287:Zinc Sulfate; D003300:Copper; D002784:Cholesterol; D010396:Penicillamine