Influence of plasma phytanic acid levels in Refsum's disease on the behaviour of the erythrocyte membrane sodium-lithium countertransporter.

Abstract

BACKGROUND:

Abnormal behaviour of the erythrocyte membrane sodium-lithium countertransporter (SLC) is associated with plasma triglyceride concentrations. Refsum's disease is characterized by progressive neurological dysfunction and accumulation of phytanic acid, an isoprenoid fatty acid, in fat-containing tissues.

METHODS:

This study explored the effects of plasma phytanic acid on SLC kinetics in nine Caucasian patients with Refsum's disease and in age- and sex-matched Caucasian control subjects.

RESULTS:

A dose-dependent association was seen between countertransporter maximal velocity and phytanic acid content of low-density lipoprotein (LDL)-cholesterol (r = -0.61, r = -0.65 respectively; P = 0.05, P = 0.04) and high-density lipoprotein (HDL)-cholesterol (r = -0.81, -0.82 respectively; P = 0.005, P = 0.003). No significant association was seen with the sodium affinity of the transporter (r = -0.44, P = 0.20, for LDL; and -0.43, P = 0.21, for high-density lipoprotein).

CONCLUSION:

These findings suggest that phytanic acid may alter the behaviour of the sodium-lithium countertransporter.

• Authors: A S Wierzbicki, T C Hardman, P Lumb, A Sankaralingam, Z Morrish, F Patel, M C Sidey, F B Gibberd
• Journal: European journal of clinical investigation (Eur J Clin Invest) Vol. 28 Issue 4 Pg. 334-8 (Apr 1998) ISSN: 0014-2972 [Print] England
• PMID: 9615914 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Antiporters
• Apolipoprotein A-I
• Apolipoprotein A-II
• Apolipoproteins B
• Cholesterol, HDL
• Cholesterol, LDL
• Fatty Acids, Nonesterified
• Lipoproteins
• Triglycerides
• sodium-lithium countertransporter
• Phytanic Acid
• Lithium
• Sodium

Topics

• Antiporters (blood)
• Apolipoprotein A-I (blood)
• Apolipoprotein A-II (blood)
• Apolipoproteins B (blood)
• Cholesterol, HDL (blood)
• Cholesterol, LDL (blood)
• Erythrocyte Membrane (metabolism)
• Fatty Acids, Nonesterified (blood)
• Female
• Humans
• Kinetics
• Lipoproteins (blood)
• Lithium (blood)
• Male
• Phytanic Acid (blood)
• Reference Values
• Refsum Disease (blood)
• Regression Analysis
• Sodium (blood)
• Triglycerides (blood)