Decreased activities in mitochondrial inner membrane electron transport system in muscle from patients with Kearns-Sayre syndrome

The present study shows biochemical data on skeletal muscle from 5 patients with Kearns-Sayre syndrome (KSS). Enzyme activities per muscle wet weight in the electron transport system of inner mitochondrial membrane were not significantly different in KSS from those in normal subjects except one pati...

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Bibliographic Details
Published inJournal of the neurological sciences Vol. 71; no. 1; pp. 65 - 75
Main Authors Yorifuji, Shiro, Ogasahara, Saburo, Takahashi, Mitsuo, Tarui, Seiichiro
Format Journal Article
LanguageEnglish
Published Shannon Elsevier B.V 01.11.1985
Elsevier Science
Subjects
Adolescent
Adult
Biological and medical sciences
Diseases of striated muscles. Neuromuscular diseases
Electron Transport
Electron transport system
Female
Histocytochemistry
Humans
inner membranes
Intracellular Membranes - enzymology
Kearns-Sayre syndrome
Kearns-Sayre Syndrome - enzymology
Kearns-Sayre Syndrome - pathology
Male
man
Medical sciences
mitochondria
Mitochondria, Muscle - enzymology
Mitochondrial inner membrane
Mitochondrial protein
Muscles - pathology
Neurology
Ophthalmoplegia - enzymology
Oxidoreductases - metabolism
skeletal muscle
Mitochondrial protein
Electron transport system
Mitochondrial inner membrane
Kearns-Sayre syndrome
Human
Intracellular transport
Mitochondria
Eye disease
Nervous system diseases
Deficiency
Ophthalmoplegia
Kearns Sayre syndrome
Mitochondrial myopathy
Electrons
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Summary:The present study shows biochemical data on skeletal muscle from 5 patients with Kearns-Sayre syndrome (KSS). Enzyme activities per muscle wet weight in the electron transport system of inner mitochondrial membrane were not significantly different in KSS from those in normal subjects except one patient with long duration of symptoms. On the other hand, mitochondrial contents were increased and enzyme activities per mitochondrial protein in the electron transport system were markedly decreased in the muscle of all cases. These results suggest that the decreased enzyme activity of the mitochondrial electron transport system in each mitochondrion may result in a compensatory increase in mitochondrial contents in the muscle of KSS.
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ISSN:0022-510X
1878-5883
DOI:10.1016/0022-510X(85)90037-1