Mitochondrial abnormalities in progressive external ophthalmoplegia

Barry Hyman; Bernard M. Patten; Ronald F. Dodson

doi:10.1016/0002-9394(77)90735-8

Mitochondrial abnormalities in progressive external ophthalmoplegia

Barry Hyman, Bernard M. Patten, Ronald F. Dodson

Academic Institute

Research output: Contribution to journal › Article › peer-review

24 Scopus citations

Abstract

Three cases of chronic progressive external ophthalmoplegia demonstrated ragged-red fibers. Histochemical stains showed that the ragged-red fiber appearance was produced by abnormal mitochondria in the muscle. Electron microscopic studies demonstrated the ultrastructural origin of the intramitochondrial inclusions seen in ragged-red fibers. The associated abnormalities in lactic acid metabolism possibly represent an underlying generalized defect in metabolism. This evidence suggests that chronic progressive external ophthalmoplegia is a diffuse disease of the mitochondria.

Original language	English (US)
Pages (from-to)	362-365,367-371
Journal	American Journal of Ophthalmology
Volume	83
Issue number	3
DOIs	https://doi.org/10.1016/0002-9394(77)90735-8
State	Published - Mar 1977

ASJC Scopus subject areas

Ophthalmology

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10.1016/0002-9394(77)90735-8

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@article{e128ca818eda483897b2a5d160435508,

title = "Mitochondrial abnormalities in progressive external ophthalmoplegia",

abstract = "Three cases of chronic progressive external ophthalmoplegia demonstrated ragged-red fibers. Histochemical stains showed that the ragged-red fiber appearance was produced by abnormal mitochondria in the muscle. Electron microscopic studies demonstrated the ultrastructural origin of the intramitochondrial inclusions seen in ragged-red fibers. The associated abnormalities in lactic acid metabolism possibly represent an underlying generalized defect in metabolism. This evidence suggests that chronic progressive external ophthalmoplegia is a diffuse disease of the mitochondria.",

author = "Barry Hyman and Patten, {Bernard M.} and Dodson, {Ronald F.}",

note = "Funding Information: From the Departments of Ophthalmology and Medicine (Dr. Hyman) and Neurology (Drs. Patten and Dodson), Baylor College of Medicine, Houston, Texas. This study was supported by a grant from the Muscular Dystrophy Association of America. Copyright: Copyright 2018 Elsevier B.V., All rights reserved.",

year = "1977",

month = mar,

doi = "10.1016/0002-9394(77)90735-8",

language = "English (US)",

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journal = "American Journal of Ophthalmology",

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T1 - Mitochondrial abnormalities in progressive external ophthalmoplegia

AU - Hyman, Barry

AU - Patten, Bernard M.

AU - Dodson, Ronald F.

N1 - Funding Information: From the Departments of Ophthalmology and Medicine (Dr. Hyman) and Neurology (Drs. Patten and Dodson), Baylor College of Medicine, Houston, Texas. This study was supported by a grant from the Muscular Dystrophy Association of America. Copyright: Copyright 2018 Elsevier B.V., All rights reserved.

PY - 1977/3

Y1 - 1977/3

N2 - Three cases of chronic progressive external ophthalmoplegia demonstrated ragged-red fibers. Histochemical stains showed that the ragged-red fiber appearance was produced by abnormal mitochondria in the muscle. Electron microscopic studies demonstrated the ultrastructural origin of the intramitochondrial inclusions seen in ragged-red fibers. The associated abnormalities in lactic acid metabolism possibly represent an underlying generalized defect in metabolism. This evidence suggests that chronic progressive external ophthalmoplegia is a diffuse disease of the mitochondria.

AB - Three cases of chronic progressive external ophthalmoplegia demonstrated ragged-red fibers. Histochemical stains showed that the ragged-red fiber appearance was produced by abnormal mitochondria in the muscle. Electron microscopic studies demonstrated the ultrastructural origin of the intramitochondrial inclusions seen in ragged-red fibers. The associated abnormalities in lactic acid metabolism possibly represent an underlying generalized defect in metabolism. This evidence suggests that chronic progressive external ophthalmoplegia is a diffuse disease of the mitochondria.

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JO - American Journal of Ophthalmology

JF - American Journal of Ophthalmology

IS - 3

ER -

Mitochondrial abnormalities in progressive external ophthalmoplegia

Abstract

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