title: Abnormal plasma lipids of patients with Retinitis pigmentosa.
abstract: 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 (22:6 omega 3) is found in high concentration in the rod outer segment membranes of the retina. Previous reports of low 22:6 omega 3 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 22:6 omega 3, 22:5 omega 3, total omega 3, 22:5 omega 6, 22:4 omega 6 and total omega 6 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 omega 6 PUFA. Evidence is accumulating mulating that RP is associated with abnormal PUFA and lipid metabolism.
authors.affiliation: Hormel Institute, University of Minnesota, Austin 55912.
authors.forename: R T
authors.initials: RT
authors.lastname: Holman
authors.forename: D M
authors.initials: DM
authors.lastname: Bibus
authors.forename: G H
authors.initials: GH
authors.lastname: Jeffrey
authors.forename: P
authors.initials: P
authors.lastname: Smethurst
authors.forename: J W
authors.initials: JW
authors.lastname: Crofts
mesh_terms: D002788:Cholesterol Esters / Q000097:blood / Q000737:chemistry; D005227:Fatty Acids / Q000032:analysis; D005230:Fatty Acids, Nonesterified / Q000097:blood; D006801:Humans; D008055:Lipids / Q000097:blood* / Q000737:chemistry; D010743:Phospholipids / Q000097:blood / Q000737:chemistry; D012174:Retinitis Pigmentosa / Q000378:metabolism*; D014280:Triglycerides / Q000097:blood / Q000737:chemistry
chemical_list: D002788:Cholesterol Esters; D005227:Fatty Acids; D005230:Fatty Acids, Nonesterified; D008055:Lipids; D010743:Phospholipids; D014280:Triglycerides