1990 Fiscal Year Final Research Report Summary
Mitochondrial Abnormalities in Ocular Diseases
| Project/Area Number |
63480396
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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 Kagoshima University Faculty of Medicine, Professor, 医学部, 教授 (50010070)
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| Co-Investigator(Kenkyū-buntansha) |
UNOKI Kazuhiko Kagoshima University Faculty of Medicine, Assistant Professor, 医学部・附属病院, 助手 (60193926)
ISASHIKI Yasushi Kagoshima University Faculty of Medicine, Assistant Professor, 医学部, 助手 (70168160)
SAMESHIMA Munefumi Kagoshima University Faculty of Medicine, Assistant Professor, 医学部, 助手 (80041333)
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| Project Period (FY) |
1988 – 1990
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| Keywords | Mitochondria / Respiratory chain enzymes / Monoclinal antibody / Retina / Myotonic dystrophy / Retinitis pigmentosa / Mitochondrial DNA / Leber's disease |
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| Research Abstract |
Basic and clinical studies were carried out for understanding mitochondrial abnormalities in ocular disorders, and the followings are summarized results.
1. A monoclinal antibody against subunit V of cytochrome c oxidase was newly prepared. Histochemical studies revealed localization of the antigen on the monkey retina.
2. Assay of Activity of the respiratory chain enzymes in the monkey retina showed predominance in the macular area, conforming to the clinical observations that the macula is more involved by mitochondrial diseases.
3. Mitochondrial abnormalities were shown in extraocular muscles of patients with myotonic dystrophy at the electron microscopic level and respiratory chain enzyme activities of extraocular muscle biopsies.
4. Deletion of mitochondrial DNA was found in skeletal muscles from patients with Kearns-Sayre syndrome associated retinal degeneration. The Wallace mutation of mitochondrial DNA was confirmed in restriction sites for SfaNI and MaeIII endonuclease cleavages in a pedigree of Leber's hereditary optic neuropathy. Deletion of mitochondrial DNA was not found in patients with sporadic cases of isolated retinitis pigmentosa.
5. Oral administration of coenzyme Q^<10> appeared to improve the central visual function in patients with retinitis pigmentosa.
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