As. Wierzbicki et al., INFLUENCE OF PLASMA PHYTANIC ACID LEVELS IN REFSUMS-DISEASE ON THE BEHAVIOR OF THE ERYTHROCYTE-MEMBRANE SODIUM-LITHIUM COUNTERTRANSPORTER, European journal of clinical investigation, 28(4), 1998, pp. 334-338
Citations number
24
Categorie Soggetti
Medicine, Research & Experimental","Medicine, General & Internal
Background Abnormal behaviour of the erythrocyte membrane sodium-lithi
um counter-transporter (SLC) is associated with plasma triglyceride co
ncentrations. Refsum's disease is characterized by progressive neurolo
gical dysfunction and accumulation of phytanic acid, an isoprenoid fat
ty acid, in fat-containing tissues. Methods This study explored the ef
fects of plasma phytanic acid on SLC kinetics in nine Caucasian patien
ts with Refsum's disease and in age-and sex-matched Caucasian control
subjects. Results A dose-dependent association was seen between counte
rtransporter 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, -0.8
2 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, f
or 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.