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Abnormal plasma lipids of patients with Retinitis pigmentosa
Author(s) -
Holman R. T.,
Bibus D. M.,
Jeffrey G. H.,
Smethurst P.,
Crofts J. W.
Publication year - 1994
Publication title -
lipids
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.601
H-Index - 120
eISSN - 1558-9307
pISSN - 0024-4201
DOI - 10.1007/bf02537092
Subject(s) - lipidology , retinitis pigmentosa , clinical chemistry , ophthalmology , chemistry , chromatography , medicine , retinal
Retinitis pigmentosa (RP) is a hereditary retinal degeneration of unknown etiology, resulting in progressive night blindness, loss of peripheral vision, abnormal retinal pigmentation and reduced electroretinographic response. Docosahexaenoic acid (22∶6ω3) is found in high concentration in the rod outer segment membranes of the retina. Previous reports of low 22∶6ω3 in blood lipids or phospholipids in RP patients prompted us to evaluate the complete fatty acid (FA) profiles of plasma phospholipids (PL), cholesteryl esters, triglycerides (TG) and nonesterified fatty acids (NEFA) in ten patients with RP. In the PL fraction, we found significantly depressed levels of 22∶6ω3, 22∶5ω3, total ω3, 22∶5ω6, 22∶4ω6 and total ω6 polyunsaturated FA (PUFA), and elevated total saturated acids. Plasma TG showed normal levels of PUFA, normal total saturated FA and total monounsaturated FA. The NEFA fraction showed significant elevation in total saturated FA with depressed total ω6 PUFA. Evidence is accumulating that RP is associated with abnormal PUFA and lipid metabolism.