TREATMENT OF EXPERIMENTAL NADH UBIQUINONE REDUCTASE DEFICIENCY WITH MENADIONE

Chronic administration of diphenylene iodonium (DPI) to rats has been shown to model the characteristics of mitochondrial myopathy. Using this model the efficacy of menadione therapy has been assessed. Menadione treatment of rats injected with DPI was associated with improved weight gain and increas...

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Veröffentlicht in:Brain (London, England : 1878) England : 1878), 1992-08, Vol.115 (4), p.991-1000
Hauptverfasser: COOPER, J. M., HAYES, D. J., CHALLISS, R. A. J., MORGAN-HUGHES, J. A., CLARK, J. B.
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container_end_page 1000
container_issue 4
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container_title Brain (London, England : 1878)
container_volume 115
creator COOPER, J. M.
HAYES, D. J.
CHALLISS, R. A. J.
MORGAN-HUGHES, J. A.
CLARK, J. B.
description Chronic administration of diphenylene iodonium (DPI) to rats has been shown to model the characteristics of mitochondrial myopathy. Using this model the efficacy of menadione therapy has been assessed. Menadione treatment of rats injected with DPI was associated with improved weight gain and increased survival rate This was accompanied by an improvement in muscle function as judged by analysis of isometric twitch tension of the gastrocnemius muscle (1 Hz for 20 min). The decline in phosphocreatine (PCr) levels in the gastrocnemius muscle during stimulation and delayed recovery in PCr after stimulation were similar in the menadione treated and untreated models Menadione treatment of the DPI model resulted in a resting intramuscular pH significantly lower than control or untreated DPI rats, but a similar decline in intramuscular pH to the DPI rats during stimulation. The changes in metabolite levels were broadly similar in both the menadione treated and untreated DPI models following stimulation, although the changes, except for increased lactate concentration, were generally less marked in the menadione-treated DPI model
doi_str_mv 10.1093/brain/115.4.991
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The decline in phosphocreatine (PCr) levels in the gastrocnemius muscle during stimulation and delayed recovery in PCr after stimulation were similar in the menadione treated and untreated models Menadione treatment of the DPI model resulted in a resting intramuscular pH significantly lower than control or untreated DPI rats, but a similar decline in intramuscular pH to the DPI rats during stimulation. 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subjects Adenosine Diphosphate - metabolism
Adenosine Triphosphate - metabolism
Animals
Biological and medical sciences
Biphenyl Compounds - administration & dosage
Biphenyl Compounds - therapeutic use
Drug Therapy, Combination
Electron Transport
Electron Transport Complex I
Hindlimb
Magnetic Resonance Spectroscopy
Male
Medical sciences
Mitochondrial Myopathies - drug therapy
Mitochondrial Myopathies - metabolism
Muscle
Muscles - metabolism
NADH, NADPH Oxidoreductases - deficiency
Onium Compounds - administration & dosage
Onium Compounds - therapeutic use
Pharmacology. Drug treatments
Phosphates - metabolism
Phosphocreatine - metabolism
Rats
Rats, Wistar
Vitamin K - administration & dosage
Vitamin K - therapeutic use
title TREATMENT OF EXPERIMENTAL NADH UBIQUINONE REDUCTASE DEFICIENCY WITH MENADIONE
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