1987 Fiscal Year Final Research Report Summary
Inversigations on the Etiology of Retinal Dystrohies
| Project/Area Number |
61480367
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| Research Category |
Grant-in-Aid for General Scientific Research (B)
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| Allocation Type | Single-year Grants |
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| Research Field |
Ophthalmology
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| Research Institution | Kagoshima University |
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Principal Investigator |
OHBA Norio Department of Ophthalmology, Faculty of Medicine, Kagoshima Univeristy, 医学部, 教授 (50010070)
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| Co-Investigator(Kenkyū-buntansha) |
UNOKI Kazuhiko Department of Ophthalmology, Faculty of Medicine, Kagoshima University, 医学部付属病院, 助手 (60193926)
冨宿 紀夫 鹿児島大学, 医学部, 助手 (40156732)
SAMESHIMA Munefumi Department of Ophthalmology, Faculty of Medicine, Kagoshima University, 医学部, 助手 (80041333)
UEHARA Fumiyuki Department of Ophthalmology, Faculty of Medicine, Kagoshima University, 医学部付属病院, 講師 (30168653)
NAGASAKO Fumiyo Department of Ophthalmology, Faculty of Medicine, Kagoshima University, 医学部付属病院, 講師 (10101180)
FUHSHUKU Norio Department of Ophthlamology, Faculty of Medicine, Kagoshima University (50010070)
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| Project Period (FY) |
1986 – 1987
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| Keywords | Retinal dystrophies / Glycoconjugates / Peanut aglutinin / Sugar transferases / Retinitis pigmentosa / Cone dystrophy / 錐体視色素 |
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| Research Abstract |
Some basic and clinical studies were carried out in order ot facilitate understanding of the features of retinal dystrophies. Peanut agglutinin, a lectin that recognizes the sugar residue galactose<beta>1-3N-acetylgalactosamine, was shown at the electron microscopic level to bind preferentially to the plasma membranes of the cone photoreceptors and cone-associated interphotoreceptor matrices of the monkey retina. The preferential PNA binding to the cone photoreceptor was found not only in the peripheral retina in which cones are intemingled by roes but also in the central retina in which the cone photoreceptor resembles in morphology the peripheral rod photoreceptor. A biochemical analysis of fucosyltransferase, galactosyltransferase and sialytransferase activities in bovine eyeballs revealed relatively high activities in the retina, with some dominant enzyme activities in the macular retion of the retina. Injections of various lectins into the subretinal space of monkey of rat retinas induced toxic or degenerative changes of the retina, the site of major involvement depending upon the type of lectin injected.
Clinically, we measured sialyltransferase and galactosyltransferase activites of the peripheral lymphocytes in patients with classical retinitis pigmentosa, and found that the sialyltransferase activities are significanly low as compared with the mormal, with no alteration of galactoryltransferase activities.
Selected patients with cone dystrophy showed in the serum antibodies sgainst central nervous system-derived proteins. A familial early-onset retinitis pigmentosa, Leber's congenital amaurosis, is described. Some psychophysical experiments with particular reference to the optical density of cone photoreceptor were cerried out on patients with retinal dystrophies and various retinal diseases.
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