Polyunsaturated fatty acids are lower in blood lipids of Usher's type I but not Usher's type II.
OBJECTIVE
Previous studies have shown that persons with retinitis pigmentosa and Usher's syndrome have lower blood levels of long-chain polyunsaturated fatty acids (PUFAs). In this study, the fatty acid composition of phospholipids from plasma and red blood cells (RBCs) was compared in persons with Usher's syndrome type I; Usher's syndrome type II; or no retinal disease (control subjects).


METHODS
Blood was drawn from fasting volunteers and separated into plasma and RBCs by centrifugation. Lipids were extracted and phospholipids were obtained by thin-layer chromatography. Fatty acid methyl esters were prepared and analyzed by gas-liquid chromatography.


RESULTS
There were no differences in plasma or RBC phospholipid fatty acid composition between control subjects (n = 54) and persons with Usher's syndrome type II (n = 20). However, all 20- and 22-carbon PUFA levels from RBCs of persons with Usher's syndrome type I were lower than those from control subjects and persons with Usher's Syndrome type II. Likewise, plasma levels of 20:3n-6, 20: 5n-3, and 22:6n-3 were lower in Usher's syndrome type I compared with the control group. In contrast, plasma levels of 18:1n-9 and RBC levels of 16:0 and 18:1n-9 were higher in the group with Usher's syndrome type I.


CONCLUSIONS
Plasma and RBCs from Usher's syndrome type I, but not type II, have lower levels of long-chain PUFAs than plasma and RBCs from control subjects.
Maude
M B
MB
Department of Ophthalmology, Oklahoma Center for Neuroscience, University of Oklahoma Health Sciences Center, Oklahoma City, USA.
Anderson
E O
EO
Anderson
R E
RE
D000328:Adult; D002849:Chromatography, Gas; D002855:Chromatography, Thin Layer; D004910:Erythrocyte Membrane / Q000378:metabolism; D005231:Fatty Acids, Unsaturated / Q000097:blood*; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000097:blood* / Q000235:genetics; D006801:Humans; D008055:Lipids; D008297:Male; D012174:Retinitis Pigmentosa / Q000097:blood* / Q000235:genetics
D005231:Fatty Acids, Unsaturated; D008055:Lipids