Mitochondrial abnormalities in progressive external ophthalmoplegia
- PMID: 848542
- DOI: 10.1016/0002-9394(77)90735-8
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.
Similar articles
-
Progressive ophthalmoplegia, glycogen storage, and abnormal mitochondria.Arch Neurol. 1973 Sep;29(3):170-9. doi: 10.1001/archneur.1973.00490270052008. Arch Neurol. 1973. PMID: 4273246 No abstract available.
-
Orbicularis oculi muscle in chronic progressive external ophthalmoplegia.Arch Ophthalmol. 1980 Jun;98(6):1070-3. doi: 10.1001/archopht.1980.01020031060010. Arch Ophthalmol. 1980. PMID: 7387511
-
[A case of mitochondrial myopathy with progressive chronic ophthalmoplegia (author's transl)].Riv Patol Nerv Ment. 1977 Nov-Dec;98(6):333-46. Riv Patol Nerv Ment. 1977. PMID: 754250 Italian.
-
[Segmental dystonia and mitochondrial encephalomyopathy].Rev Neurol (Paris). 1992;148(1):51-3. Rev Neurol (Paris). 1992. PMID: 1318569 Review. French.
-
[Ocular myopathies. Nosological study of 49 cases].Presse Med. 1987 Nov 21;16(39):1969-74. Presse Med. 1987. PMID: 2962160 Review. French.
Cited by
-
Hyperglycemic acidotic coma and death in Kearns-Sayre syndrome.Trans Am Ophthalmol Soc. 1985;83:131-61. Trans Am Ophthalmol Soc. 1985. PMID: 3832524 Free PMC article. No abstract available.
-
The occurrence of paracrystalline mitochondrial inclusions in normal human skeletal muscle.Acta Neuropathol. 1980;49(1):35-41. doi: 10.1007/BF00692217. Acta Neuropathol. 1980. PMID: 6243837
-
Myopathy with mitochondrial abnormalities and rimmed vacuoles.Acta Neuropathol. 1986;70(1):86-90. doi: 10.1007/BF00689520. Acta Neuropathol. 1986. PMID: 3727938
-
Ophthalmoplegia-plus.Doc Ophthalmol. 1979 Mar 15;46(2):365-80. doi: 10.1007/BF00142628. Doc Ophthalmol. 1979. PMID: 477490 No abstract available.
-
Megamitochondria formation - physiology and pathology.J Cell Mol Med. 2002 Oct-Dec;6(4):497-538. doi: 10.1111/j.1582-4934.2002.tb00452.x. J Cell Mol Med. 2002. PMID: 12611638 Free PMC article. Review.
Publication types
MeSH terms
Substances
LinkOut - more resources
Full Text Sources