Studies of lipoprotein metabolism in a patient with fish-eye disease.

Abstract

In the rare familial disorder fish-eye disease, hypertriglyceridaemia is associated with elevated levels of very-low-density lipoprotein (VLDL) and enrichment of low-density lipoprotein (LDL) with triglyceride. The kinetic basis of the dyslipoproteinaemia was investigated by studying the metabolism of the apolipoprotein-B moeity of VLDL, intermediate-density lipoprotein (IDL) and LDL in a 68-year-old woman with this condition. The major kinetic abnormality was a pronounced reduction in the rate of fractional conversion of VLDL-B to IDL-B and of IDL-B to LDL-B, suggesting that the dyslipoproteinaemia represents accumulation in plasma of partly degraded products of VLDL metabolism. This kinetic disorder has features in common with type-III hyperlipoproteinaemia. In studies in vitro no defect in the enzyme, activator or substrate components of the lipoprotein lipase or hepatic lipase systems was observed.

• Authors: P R Turner, L A Carlson, C Cortese, S Rao, C B Marenah, N E Miller, B Lewis
• Journal: European journal of clinical investigation (Eur J Clin Invest) Vol. 14 Issue 4 Pg. 273-7 (Aug 1984) ISSN: 0014-2972 [Print] England
• PMID: 6434321 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Cholesterol, HDL
• Cholesterol, LDL
• Lipoproteins
• Lipoproteins, IDL
• Lipoproteins, VLDL
• Triglycerides
• Cholesterol

Topics

• Aged
• Cholesterol (blood)
• Cholesterol, HDL (blood)
• Cholesterol, LDL (blood)
• Eye Diseases (blood, complications)
• Female
• Humans
• Hyperlipoproteinemia Type IV (blood)
• Kinetics
• Lipoproteins (blood)
• Lipoproteins, IDL
• Lipoproteins, VLDL (blood)
• Middle Aged
• Triglycerides (blood)