ParkinsonFranceV1, Main, Curation, bibRecord, 004942

Mitochondrial respiratory failure in skeletal muscle from patients with Parkinson's disease and multiple system atrophy

Identifieur interne : 004942 ( Main/Curation ); précédent : 004941; suivant : 004943

Mitochondrial respiratory failure in skeletal muscle from patients with Parkinson's disease and multiple system atrophy

Auteurs : O. Blin [France] ; C. Desnuelle [France] ; O. Rascol [France] ; M. Borg [France] ; H. Peyro Saint Paul [France] ; J. P. Azulay [France] ; F. Billé [France] ; D. Figarella [France] ; F. Coulom [France] ; J. F. Pellissier [France] ; J. L. Montastruc [France] ; M. Chatel [France] ; G. Serratrice [France]

Source :

Journal of the Neurological Sciences [ 0022-510X ] ; 1994.

RBID : ISTEX:0369D0033894298A8324FB758ADC53C38CBF1146

Descripteurs français

Pascal (Inist)
- Atrophie multisystématisée, Chaîne respiratoire, Exploration, Homme, Mitochondrie, Muscle strié, Parkinson maladie.
Wicri :
- topic : Homme.

English descriptors

KwdEn :
- Adult, Aged, Aged, 80 and over, Atrophy, Brain Diseases (metabolism), Brain Diseases (pathology), Electron Transport Complex I, Electron Transport Complex III (metabolism), Electron Transport Complex IV (metabolism), Exploration, Histocytochemistry, Human, Humans, Middle Aged, Mitochondria, Mitochondria, Muscle (metabolism), Multiple system atrophy, Muscle, Skeletal (metabolism), NAD(P)H Dehydrogenase (Quinone) (metabolism), NADH, NADPH Oxidoreductases (metabolism), Nerve Degeneration, Oxygen Consumption, Parkinson Disease (metabolism), Parkinson disease, Regression Analysis, Respiratory chain, Striated muscle.
MESH :
- chemical , metabolism : Electron Transport Complex III, Electron Transport Complex IV, NAD(P)H Dehydrogenase (Quinone), NADH, NADPH Oxidoreductases.
- chemical : Electron Transport Complex I.
- metabolism : Brain Diseases, Mitochondria, Muscle, Muscle, Skeletal, Parkinson Disease.
- pathology : Brain Diseases.
- Adult, Aged, Aged, 80 and over, Atrophy, Histocytochemistry, Humans, Middle Aged, Nerve Degeneration, Oxygen Consumption, Regression Analysis.

Abstract

We studied mitochondrial respiratory chain function in skeletal muscle taken from 27 patients with idiopathic Parkinson's disease (PD; 21 Dopa-treated PD patients and 6 de novo patients), 5 patients with multiple system atrophy (MSA) and from 43 age-matched controls in order to determine the occurrence of mitochondrial respiratory chain abnormalities in parkinsonian syndromes. In our control subjects, we found a significant age-related decrease in the activity of respiratory chain complex I. As compared to carefully age-matched control subjects, activity of complex (NADH: ubiquinone reductase) was significantly lower in muscle mitochondria from patients with PD and MSA and a mean remaining activity < 30% of controls was observed. Mean activities of complexes III (ubiquinol:cytochrome c reductase) and IV (cytochrome c oxidase) were also lower in PD patients than controls, but a low activity (remaining activity < 30% of controls) was observed in only 5 PD patients for complex I and III or I and IV. No deficit in complex II activity (succinate: ubiquinone reductase) was observed. Our results support the hypothesis of a wide-spread mitochondrial complex I deficiency in PD and MSA as compared to age-matched controls, who showed age-related deficiency. This deficit can be found in de novo PD patients as well as in treated patients. The observed respiratory enzyme chain deficiency could not be explained by the dose and duration of l-Dopa or dopaminergic agonist treatment, the severity of the disease, anxiety or depression since no significant correlation was found between these parameters and enzyme complexes activities.

Url:

https://api.istex.fr/document/0369D0033894298A8324FB758ADC53C38CBF1146/fulltext/pdf

DOI: 10.1016/0022-510X(94)90248-8

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ISTEX:0369D0033894298A8324FB758ADC53C38CBF1146

Le document en format XML

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<term>Aged</term>
<term>Aged, 80 and over</term>
<term>Atrophy</term>
<term>Brain Diseases (metabolism)</term>
<term>Brain Diseases (pathology)</term>
<term>Electron Transport Complex I</term>
<term>Electron Transport Complex III (metabolism)</term>
<term>Electron Transport Complex IV (metabolism)</term>
<term>Exploration</term>
<term>Histocytochemistry</term>
<term>Human</term>
<term>Humans</term>
<term>Middle Aged</term>
<term>Mitochondria</term>
<term>Mitochondria, Muscle (metabolism)</term>
<term>Multiple system atrophy</term>
<term>Muscle, Skeletal (metabolism)</term>
<term>NAD(P)H Dehydrogenase (Quinone) (metabolism)</term>
<term>NADH, NADPH Oxidoreductases (metabolism)</term>
<term>Nerve Degeneration</term>
<term>Oxygen Consumption</term>
<term>Parkinson Disease (metabolism)</term>
<term>Parkinson disease</term>
<term>Regression Analysis</term>
<term>Respiratory chain</term>
<term>Striated muscle</term>
</keywords>
<keywords scheme="MESH" type="chemical" qualifier="metabolism" xml:lang="en"><term>Electron Transport Complex III</term>
<term>Electron Transport Complex IV</term>
<term>NAD(P)H Dehydrogenase (Quinone)</term>
<term>NADH, NADPH Oxidoreductases</term>
</keywords>
<keywords scheme="MESH" type="chemical" xml:lang="en"><term>Electron Transport Complex I</term>
</keywords>
<keywords scheme="MESH" qualifier="metabolism" xml:lang="en"><term>Brain Diseases</term>
<term>Mitochondria, Muscle</term>
<term>Muscle, Skeletal</term>
<term>Parkinson Disease</term>
</keywords>
<keywords scheme="MESH" qualifier="pathology" xml:lang="en"><term>Brain Diseases</term>
</keywords>
<keywords scheme="MESH" xml:lang="en"><term>Adult</term>
<term>Aged</term>
<term>Aged, 80 and over</term>
<term>Atrophy</term>
<term>Histocytochemistry</term>
<term>Humans</term>
<term>Middle Aged</term>
<term>Nerve Degeneration</term>
<term>Oxygen Consumption</term>
<term>Regression Analysis</term>
</keywords>
<keywords scheme="Pascal" xml:lang="fr"><term>Atrophie multisystématisée</term>
<term>Chaîne respiratoire</term>
<term>Exploration</term>
<term>Homme</term>
<term>Mitochondrie</term>
<term>Muscle strié</term>
<term>Parkinson maladie</term>
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<front><div type="abstract" xml:lang="en">We studied mitochondrial respiratory chain function in skeletal muscle taken from 27 patients with idiopathic Parkinson's disease (PD; 21 Dopa-treated PD patients and 6 de novo patients), 5 patients with multiple system atrophy (MSA) and from 43 age-matched controls in order to determine the occurrence of mitochondrial respiratory chain abnormalities in parkinsonian syndromes. In our control subjects, we found a significant age-related decrease in the activity of respiratory chain complex I. As compared to carefully age-matched control subjects, activity of complex (NADH: ubiquinone reductase) was significantly lower in muscle mitochondria from patients with PD and MSA and a mean remaining activity < 30% of controls was observed. Mean activities of complexes III (ubiquinol:cytochrome c reductase) and IV (cytochrome c oxidase) were also lower in PD patients than controls, but a low activity (remaining activity < 30% of controls) was observed in only 5 PD patients for complex I and III or I and IV. No deficit in complex II activity (succinate: ubiquinone reductase) was observed. Our results support the hypothesis of a wide-spread mitochondrial complex I deficiency in PD and MSA as compared to age-matched controls, who showed age-related deficiency. This deficit can be found in de novo PD patients as well as in treated patients. The observed respiratory enzyme chain deficiency could not be explained by the dose and duration of l-Dopa or dopaminergic agonist treatment, the severity of the disease, anxiety or depression since no significant correlation was found between these parameters and enzyme complexes activities.</div>
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<s3>FRA</s3>
<sZ>6 aut.</sZ>
<sZ>7 aut.</sZ>
<sZ>9 aut.</sZ>
<sZ>13 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName><region type="region" nuts="2">Provence-Alpes-Côte d'Azur</region>
<settlement type="city">Marseille</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Figarella, D" sort="Figarella, D" uniqKey="Figarella D" first="D." last="Figarella">D. Figarella</name>
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<s2>13385 Marseille</s2>
<s3>FRA</s3>
<sZ>8 aut.</sZ>
<sZ>10 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName><region type="region" nuts="2">Provence-Alpes-Côte d'Azur</region>
<settlement type="city">Marseille</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Coulom, F" sort="Coulom, F" uniqKey="Coulom F" first="F." last="Coulom">F. Coulom</name>
<affiliation wicri:level="3"><inist:fA14 i1="02"><s1>CHU Timone, clin. maladies systèmes nerveux appareil locomoteur</s1>
<s2>13385 Marseille</s2>
<s3>FRA</s3>
<sZ>6 aut.</sZ>
<sZ>7 aut.</sZ>
<sZ>9 aut.</sZ>
<sZ>13 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName><region type="region" nuts="2">Provence-Alpes-Côte d'Azur</region>
<settlement type="city">Marseille</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Pellissier, J F" sort="Pellissier, J F" uniqKey="Pellissier J" first="J. F." last="Pellissier">J. F. Pellissier</name>
<affiliation wicri:level="3"><inist:fA14 i1="03"><s1>CHU Timone, dép. neuropathologie</s1>
<s2>13385 Marseille</s2>
<s3>FRA</s3>
<sZ>8 aut.</sZ>
<sZ>10 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName><region type="region" nuts="2">Provence-Alpes-Côte d'Azur</region>
<settlement type="city">Marseille</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Montastruc, J L" sort="Montastruc, J L" uniqKey="Montastruc J" first="J. L." last="Montastruc">J. L. Montastruc</name>
</author>
<author><name sortKey="Chatel, M" sort="Chatel, M" uniqKey="Chatel M" first="M." last="Chatel">M. Chatel</name>
</author>
<author><name sortKey="Serratrice, G" sort="Serratrice, G" uniqKey="Serratrice G" first="G." last="Serratrice">G. Serratrice</name>
<affiliation wicri:level="3"><inist:fA14 i1="02"><s1>CHU Timone, clin. maladies systèmes nerveux appareil locomoteur</s1>
<s2>13385 Marseille</s2>
<s3>FRA</s3>
<sZ>6 aut.</sZ>
<sZ>7 aut.</sZ>
<sZ>9 aut.</sZ>
<sZ>13 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName><region type="region" nuts="2">Provence-Alpes-Côte d'Azur</region>
<settlement type="city">Marseille</settlement>
</placeName>
</affiliation>
</author>
</titleStmt>
<publicationStmt><idno type="wicri:source">INIST</idno>
<idno type="inist">94-0624765</idno>
<date when="1994">1994</date>
<idno type="stanalyst">PASCAL 94-0624765 INIST</idno>
<idno type="RBID">Pascal:94-0624765</idno>
<idno type="wicri:Area/PascalFrancis/Corpus">001948</idno>
<idno type="wicri:Area/PascalFrancis/Curation">001704</idno>
<idno type="wicri:Area/PascalFrancis/Checkpoint">001795</idno>
<idno type="wicri:explorRef" wicri:stream="PascalFrancis" wicri:step="Checkpoint">001795</idno>
<idno type="wicri:doubleKey">0022-510X:1994:Blin O:mitochondrial:respiratory:failure</idno>
<idno type="wicri:Area/Main/Merge">005439</idno>
</publicationStmt>
<sourceDesc><biblStruct><analytic><title xml:lang="en" level="a">Mitochondrial respiratory failure in skeletal muscle from patients with Parkinson's disease and multiple system atrophy</title>
<author><name sortKey="Blin, O" sort="Blin, O" uniqKey="Blin O" first="O." last="Blin">O. Blin</name>
<affiliation wicri:level="3"><inist:fA14 i1="01"><s1>CHU Timone, pharmacologie médicale clin. CPCET</s1>
<s2>13385 Marseille</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName><region type="region" nuts="2">Provence-Alpes-Côte d'Azur</region>
<settlement type="city">Marseille</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Desnuelle, C" sort="Desnuelle, C" uniqKey="Desnuelle C" first="C." last="Desnuelle">C. Desnuelle</name>
</author>
<author><name sortKey="Rascol, O" sort="Rascol, O" uniqKey="Rascol O" first="O." last="Rascol">O. Rascol</name>
</author>
<author><name sortKey="Borg, M" sort="Borg, M" uniqKey="Borg M" first="M." last="Borg">M. Borg</name>
</author>
<author><name sortKey="Peyro Saint Paul, H" sort="Peyro Saint Paul, H" uniqKey="Peyro Saint Paul H" first="H." last="Peyro Saint Paul">H. Peyro Saint Paul</name>
</author>
<author><name sortKey="Azulay, J P" sort="Azulay, J P" uniqKey="Azulay J" first="J. P." last="Azulay">J. P. Azulay</name>
<affiliation wicri:level="3"><inist:fA14 i1="02"><s1>CHU Timone, clin. maladies systèmes nerveux appareil locomoteur</s1>
<s2>13385 Marseille</s2>
<s3>FRA</s3>
<sZ>6 aut.</sZ>
<sZ>7 aut.</sZ>
<sZ>9 aut.</sZ>
<sZ>13 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName><region type="region" nuts="2">Provence-Alpes-Côte d'Azur</region>
<settlement type="city">Marseille</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Bille, F" sort="Bille, F" uniqKey="Bille F" first="F." last="Bille">F. Bille</name>
<affiliation wicri:level="3"><inist:fA14 i1="02"><s1>CHU Timone, clin. maladies systèmes nerveux appareil locomoteur</s1>
<s2>13385 Marseille</s2>
<s3>FRA</s3>
<sZ>6 aut.</sZ>
<sZ>7 aut.</sZ>
<sZ>9 aut.</sZ>
<sZ>13 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName><region type="region" nuts="2">Provence-Alpes-Côte d'Azur</region>
<settlement type="city">Marseille</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Figarella, D" sort="Figarella, D" uniqKey="Figarella D" first="D." last="Figarella">D. Figarella</name>
<affiliation wicri:level="3"><inist:fA14 i1="03"><s1>CHU Timone, dép. neuropathologie</s1>
<s2>13385 Marseille</s2>
<s3>FRA</s3>
<sZ>8 aut.</sZ>
<sZ>10 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName><region type="region" nuts="2">Provence-Alpes-Côte d'Azur</region>
<settlement type="city">Marseille</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Coulom, F" sort="Coulom, F" uniqKey="Coulom F" first="F." last="Coulom">F. Coulom</name>
<affiliation wicri:level="3"><inist:fA14 i1="02"><s1>CHU Timone, clin. maladies systèmes nerveux appareil locomoteur</s1>
<s2>13385 Marseille</s2>
<s3>FRA</s3>
<sZ>6 aut.</sZ>
<sZ>7 aut.</sZ>
<sZ>9 aut.</sZ>
<sZ>13 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName><region type="region" nuts="2">Provence-Alpes-Côte d'Azur</region>
<settlement type="city">Marseille</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Pellissier, J F" sort="Pellissier, J F" uniqKey="Pellissier J" first="J. F." last="Pellissier">J. F. Pellissier</name>
<affiliation wicri:level="3"><inist:fA14 i1="03"><s1>CHU Timone, dép. neuropathologie</s1>
<s2>13385 Marseille</s2>
<s3>FRA</s3>
<sZ>8 aut.</sZ>
<sZ>10 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName><region type="region" nuts="2">Provence-Alpes-Côte d'Azur</region>
<settlement type="city">Marseille</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Montastruc, J L" sort="Montastruc, J L" uniqKey="Montastruc J" first="J. L." last="Montastruc">J. L. Montastruc</name>
</author>
<author><name sortKey="Chatel, M" sort="Chatel, M" uniqKey="Chatel M" first="M." last="Chatel">M. Chatel</name>
</author>
<author><name sortKey="Serratrice, G" sort="Serratrice, G" uniqKey="Serratrice G" first="G." last="Serratrice">G. Serratrice</name>
<affiliation wicri:level="3"><inist:fA14 i1="02"><s1>CHU Timone, clin. maladies systèmes nerveux appareil locomoteur</s1>
<s2>13385 Marseille</s2>
<s3>FRA</s3>
<sZ>6 aut.</sZ>
<sZ>7 aut.</sZ>
<sZ>9 aut.</sZ>
<sZ>13 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName><region type="region" nuts="2">Provence-Alpes-Côte d'Azur</region>
<settlement type="city">Marseille</settlement>
</placeName>
</affiliation>
</author>
</analytic>
<series><title level="j" type="main">Journal of the neurological sciences</title>
<title level="j" type="abbreviated">J. neurol. sci.</title>
<idno type="ISSN">0022-510X</idno>
<imprint><date when="1994">1994</date>
</imprint>
</series>
</biblStruct>
</sourceDesc>
<seriesStmt><title level="j" type="main">Journal of the neurological sciences</title>
<title level="j" type="abbreviated">J. neurol. sci.</title>
<idno type="ISSN">0022-510X</idno>
</seriesStmt>
</fileDesc>
<profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Exploration</term>
<term>Human</term>
<term>Mitochondria</term>
<term>Multiple system atrophy</term>
<term>Parkinson disease</term>
<term>Respiratory chain</term>
<term>Striated muscle</term>
</keywords>
<keywords scheme="Pascal" xml:lang="fr"><term>Parkinson maladie</term>
<term>Chaîne respiratoire</term>
<term>Mitochondrie</term>
<term>Muscle strié</term>
<term>Exploration</term>
<term>Homme</term>
<term>Atrophie multisystématisée</term>
</keywords>
<keywords scheme="Wicri" type="topic" xml:lang="fr"><term>Homme</term>
</keywords>
</textClass>
</profileDesc>
</teiHeader>
<front><div type="abstract" xml:lang="en">We studied mitochondrial respiratory chain function in skeletal muscle taken from 27 patients with idiopathic Parkinson's disease (PD; 21 Dopa-treated PD patients and 6 de novo patients), 5 patients with multiple system atrophy (MSA) and from 43 age-matched controls in order to determine the occurrence of mitochondrial respiratory chain abnormalities in parkinsonian syndromes. In our control subjects, we found a significant age-related decrease in the activity of respiratory chain complex I. As compared to carefully age-matched control subjects, activity of complex (NADH: ubiquinone reductase) was significantly lower in muscle mitochondria from patients with PD and MSA and a mean remaining activity <30% of controls was observed</div>
</front>
</TEI>
</INIST>
<ISTEX><TEI wicri:istexFullTextTei="biblStruct"><teiHeader><fileDesc><titleStmt><title>Mitochondrial respiratory failure in skeletal muscle from patients with Parkinson's disease and multiple system atrophy</title>
<author><name sortKey="Blin, O" sort="Blin, O" uniqKey="Blin O" first="O." last="Blin">O. Blin</name>
</author>
<author><name sortKey="Desnuelle, C" sort="Desnuelle, C" uniqKey="Desnuelle C" first="C." last="Desnuelle">C. Desnuelle</name>
</author>
<author><name sortKey="Rascol, O" sort="Rascol, O" uniqKey="Rascol O" first="O." last="Rascol">O. Rascol</name>
</author>
<author><name sortKey="Borg, M" sort="Borg, M" uniqKey="Borg M" first="M." last="Borg">M. Borg</name>
</author>
<author><name sortKey="Paul, H Peyro Saint" sort="Paul, H Peyro Saint" uniqKey="Paul H" first="H. Peyro Saint" last="Paul">H. Peyro Saint Paul</name>
</author>
<author><name sortKey="Azulay, J P" sort="Azulay, J P" uniqKey="Azulay J" first="J. P." last="Azulay">J. P. Azulay</name>
</author>
<author><name sortKey="Bille, F" sort="Bille, F" uniqKey="Bille F" first="F." last="Billé">F. Billé</name>
</author>
<author><name sortKey="Figarella, D" sort="Figarella, D" uniqKey="Figarella D" first="D." last="Figarella">D. Figarella</name>
</author>
<author><name sortKey="Coulom, F" sort="Coulom, F" uniqKey="Coulom F" first="F." last="Coulom">F. Coulom</name>
</author>
<author><name sortKey="Pellissier, J F" sort="Pellissier, J F" uniqKey="Pellissier J" first="J. F." last="Pellissier">J. F. Pellissier</name>
</author>
<author><name sortKey="Montastruc, J L" sort="Montastruc, J L" uniqKey="Montastruc J" first="J. L." last="Montastruc">J. L. Montastruc</name>
</author>
<author><name sortKey="Chatel, M" sort="Chatel, M" uniqKey="Chatel M" first="M." last="Chatel">M. Chatel</name>
</author>
<author><name sortKey="Serratrice, G" sort="Serratrice, G" uniqKey="Serratrice G" first="G." last="Serratrice">G. Serratrice</name>
</author>
</titleStmt>
<publicationStmt><idno type="wicri:source">ISTEX</idno>
<idno type="RBID">ISTEX:0369D0033894298A8324FB758ADC53C38CBF1146</idno>
<date when="1994" year="1994">1994</date>
<idno type="doi">10.1016/0022-510X(94)90248-8</idno>
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<idno type="wicri:Area/Istex/Curation">001852</idno>
<idno type="wicri:Area/Istex/Checkpoint">001991</idno>
<idno type="wicri:explorRef" wicri:stream="Istex" wicri:step="Checkpoint">001991</idno>
<idno type="wicri:doubleKey">0022-510X:1994:Blin O:mitochondrial:respiratory:failure</idno>
<idno type="wicri:Area/Main/Merge">005296</idno>
</publicationStmt>
<sourceDesc><biblStruct><analytic><title level="a">Mitochondrial respiratory failure in skeletal muscle from patients with Parkinson's disease and multiple system atrophy</title>
<author><name sortKey="Blin, O" sort="Blin, O" uniqKey="Blin O" first="O." last="Blin">O. Blin</name>
<affiliation wicri:level="3"><country xml:lang="fr">France</country>
<wicri:regionArea>Pharmacologie Médicale et Clinique and CPCET, CHU Timone, 13385 Marseille Cedex 5</wicri:regionArea>
<placeName><region type="region" nuts="2">Provence-Alpes-Côte d'Azur</region>
<settlement type="city">Marseille</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Desnuelle, C" sort="Desnuelle, C" uniqKey="Desnuelle C" first="C." last="Desnuelle">C. Desnuelle</name>
<affiliation wicri:level="3"><country xml:lang="fr">France</country>
<wicri:regionArea>Service de Neurologie, CHU Pasteur, 06000 Nice</wicri:regionArea>
<placeName><region type="region" nuts="2">Provence-Alpes-Côte d'Azur</region>
<settlement type="city">Nice</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Rascol, O" sort="Rascol, O" uniqKey="Rascol O" first="O." last="Rascol">O. Rascol</name>
<affiliation wicri:level="3"><country xml:lang="fr">France</country>
<wicri:regionArea>Laboratoire de Pharmacologie Médicale et Clinique, Faculté de Médecine, 31073 Toulouse</wicri:regionArea>
<placeName><region type="region" nuts="2">Occitanie (région administrative)</region>
<region type="old region" nuts="2">Midi-Pyrénées</region>
<settlement type="city">Toulouse</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Borg, M" sort="Borg, M" uniqKey="Borg M" first="M." last="Borg">M. Borg</name>
<affiliation wicri:level="3"><country xml:lang="fr">France</country>
<wicri:regionArea>Service de Neurologie, CHU Pasteur, 06000 Nice</wicri:regionArea>
<placeName><region type="region" nuts="2">Provence-Alpes-Côte d'Azur</region>
<settlement type="city">Nice</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Paul, H Peyro Saint" sort="Paul, H Peyro Saint" uniqKey="Paul H" first="H. Peyro Saint" last="Paul">H. Peyro Saint Paul</name>
<affiliation wicri:level="3"><country xml:lang="fr">France</country>
<wicri:regionArea>Laboratoire de Pharmacologie Médicale et Clinique, Faculté de Médecine, 31073 Toulouse</wicri:regionArea>
<placeName><region type="region" nuts="2">Occitanie (région administrative)</region>
<region type="old region" nuts="2">Midi-Pyrénées</region>
<settlement type="city">Toulouse</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Azulay, J P" sort="Azulay, J P" uniqKey="Azulay J" first="J. P." last="Azulay">J. P. Azulay</name>
<affiliation wicri:level="3"><country xml:lang="fr">France</country>
<wicri:regionArea>Clinique des Maladies du Système nerveux et de l'Appareil Locomoteur, CHU Timone, 13385 Marseille Cedex 5</wicri:regionArea>
<placeName><region type="region" nuts="2">Provence-Alpes-Côte d'Azur</region>
<settlement type="city">Marseille</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Bille, F" sort="Bille, F" uniqKey="Bille F" first="F." last="Billé">F. Billé</name>
<affiliation wicri:level="3"><country xml:lang="fr">France</country>
<wicri:regionArea>Clinique des Maladies du Système nerveux et de l'Appareil Locomoteur, CHU Timone, 13385 Marseille Cedex 5</wicri:regionArea>
<placeName><region type="region" nuts="2">Provence-Alpes-Côte d'Azur</region>
<settlement type="city">Marseille</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Figarella, D" sort="Figarella, D" uniqKey="Figarella D" first="D." last="Figarella">D. Figarella</name>
<affiliation wicri:level="3"><country xml:lang="fr">France</country>
<wicri:regionArea>Département de Neuropathologie, CHU Timone, 13385 Marseille Cedex 5</wicri:regionArea>
<placeName><region type="region" nuts="2">Provence-Alpes-Côte d'Azur</region>
<settlement type="city">Marseille</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Coulom, F" sort="Coulom, F" uniqKey="Coulom F" first="F." last="Coulom">F. Coulom</name>
<affiliation wicri:level="3"><country xml:lang="fr">France</country>
<wicri:regionArea>Clinique des Maladies du Système nerveux et de l'Appareil Locomoteur, CHU Timone, 13385 Marseille Cedex 5</wicri:regionArea>
<placeName><region type="region" nuts="2">Provence-Alpes-Côte d'Azur</region>
<settlement type="city">Marseille</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Pellissier, J F" sort="Pellissier, J F" uniqKey="Pellissier J" first="J. F." last="Pellissier">J. F. Pellissier</name>
<affiliation wicri:level="3"><country xml:lang="fr">France</country>
<wicri:regionArea>Département de Neuropathologie, CHU Timone, 13385 Marseille Cedex 5</wicri:regionArea>
<placeName><region type="region" nuts="2">Provence-Alpes-Côte d'Azur</region>
<settlement type="city">Marseille</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Montastruc, J L" sort="Montastruc, J L" uniqKey="Montastruc J" first="J. L." last="Montastruc">J. L. Montastruc</name>
<affiliation wicri:level="3"><country xml:lang="fr">France</country>
<wicri:regionArea>Laboratoire de Pharmacologie Médicale et Clinique, Faculté de Médecine, 31073 Toulouse</wicri:regionArea>
<placeName><region type="region" nuts="2">Occitanie (région administrative)</region>
<region type="old region" nuts="2">Midi-Pyrénées</region>
<settlement type="city">Toulouse</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Chatel, M" sort="Chatel, M" uniqKey="Chatel M" first="M." last="Chatel">M. Chatel</name>
<affiliation wicri:level="3"><country xml:lang="fr">France</country>
<wicri:regionArea>Service de Neurologie, CHU Pasteur, 06000 Nice</wicri:regionArea>
<placeName><region type="region" nuts="2">Provence-Alpes-Côte d'Azur</region>
<settlement type="city">Nice</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Serratrice, G" sort="Serratrice, G" uniqKey="Serratrice G" first="G." last="Serratrice">G. Serratrice</name>
<affiliation wicri:level="3"><country xml:lang="fr">France</country>
<wicri:regionArea>Clinique des Maladies du Système nerveux et de l'Appareil Locomoteur, CHU Timone, 13385 Marseille Cedex 5</wicri:regionArea>
<placeName><region type="region" nuts="2">Provence-Alpes-Côte d'Azur</region>
<settlement type="city">Marseille</settlement>
</placeName>
</affiliation>
</author>
</analytic>
<monogr></monogr>
<series><title level="j">Journal of the Neurological Sciences</title>
<title level="j" type="abbrev">JNS</title>
<idno type="ISSN">0022-510X</idno>
<imprint><publisher>ELSEVIER</publisher>
<date type="published" when="1994">1994</date>
<biblScope unit="volume">125</biblScope>
<biblScope unit="issue">1</biblScope>
<biblScope unit="page" from="95">95</biblScope>
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<front><div type="abstract" xml:lang="en">We studied mitochondrial respiratory chain function in skeletal muscle taken from 27 patients with idiopathic Parkinson's disease (PD; 21 Dopa-treated PD patients and 6 de novo patients), 5 patients with multiple system atrophy (MSA) and from 43 age-matched controls in order to determine the occurrence of mitochondrial respiratory chain abnormalities in parkinsonian syndromes. In our control subjects, we found a significant age-related decrease in the activity of respiratory chain complex I. As compared to carefully age-matched control subjects, activity of complex (NADH: ubiquinone reductase) was significantly lower in muscle mitochondria from patients with PD and MSA and a mean remaining activity < 30% of controls was observed. Mean activities of complexes III (ubiquinol:cytochrome c reductase) and IV (cytochrome c oxidase) were also lower in PD patients than controls, but a low activity (remaining activity < 30% of controls) was observed in only 5 PD patients for complex I and III or I and IV. No deficit in complex II activity (succinate: ubiquinone reductase) was observed. Our results support the hypothesis of a wide-spread mitochondrial complex I deficiency in PD and MSA as compared to age-matched controls, who showed age-related deficiency. This deficit can be found in de novo PD patients as well as in treated patients. The observed respiratory enzyme chain deficiency could not be explained by the dose and duration of l-Dopa or dopaminergic agonist treatment, the severity of the disease, anxiety or depression since no significant correlation was found between these parameters and enzyme complexes activities.</div>
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<placeName><region type="region">Provence-Alpes-Côte d'Azur</region>
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<author><name sortKey="Desnuelle, C" sort="Desnuelle, C" uniqKey="Desnuelle C" first="C" last="Desnuelle">C. Desnuelle</name>
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<author><name sortKey="Rascol, O" sort="Rascol, O" uniqKey="Rascol O" first="O" last="Rascol">O. Rascol</name>
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<author><name sortKey="Borg, M" sort="Borg, M" uniqKey="Borg M" first="M" last="Borg">M. Borg</name>
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<author><name sortKey="Peyro Saint Paul, H" sort="Peyro Saint Paul, H" uniqKey="Peyro Saint Paul H" first="H" last="Peyro Saint Paul">H. Peyro Saint Paul</name>
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<author><name sortKey="Rascol, O" sort="Rascol, O" uniqKey="Rascol O" first="O" last="Rascol">O. Rascol</name>
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<author><name sortKey="Peyro Saint Paul, H" sort="Peyro Saint Paul, H" uniqKey="Peyro Saint Paul H" first="H" last="Peyro Saint Paul">H. Peyro Saint Paul</name>
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<term>Aged</term>
<term>Aged, 80 and over</term>
<term>Atrophy</term>
<term>Brain Diseases (metabolism)</term>
<term>Brain Diseases (pathology)</term>
<term>Electron Transport Complex I</term>
<term>Electron Transport Complex III (metabolism)</term>
<term>Electron Transport Complex IV (metabolism)</term>
<term>Histocytochemistry</term>
<term>Humans</term>
<term>Middle Aged</term>
<term>Mitochondria, Muscle (metabolism)</term>
<term>Muscle, Skeletal (metabolism)</term>
<term>NAD(P)H Dehydrogenase (Quinone) (metabolism)</term>
<term>NADH, NADPH Oxidoreductases (metabolism)</term>
<term>Nerve Degeneration</term>
<term>Oxygen Consumption</term>
<term>Parkinson Disease (metabolism)</term>
<term>Regression Analysis</term>
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<term>NADH, NADPH Oxidoreductases</term>
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<keywords scheme="MESH" qualifier="metabolism" xml:lang="en"><term>Brain Diseases</term>
<term>Mitochondria, Muscle</term>
<term>Muscle, Skeletal</term>
<term>Parkinson Disease</term>
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<term>Aged</term>
<term>Aged, 80 and over</term>
<term>Atrophy</term>
<term>Histocytochemistry</term>
<term>Humans</term>
<term>Middle Aged</term>
<term>Nerve Degeneration</term>
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<front><div type="abstract" xml:lang="en">We studied mitochondrial respiratory chain function in skeletal muscle taken from 27 patients with idiopathic Parkinson's disease (PD; 21 Dopa-treated PD patients and 6 de novo patients), 5 patients with multiple system atrophy (MSA) and from 43 age-matched controls in order to determine the occurrence of mitochondrial respiratory chain abnormalities in parkinsonian syndromes. In our control subjects, we found a significant age-related decrease in the activity of respiratory chain complex I. As compared to carefully age-matched control subjects, activity of complex (NADH:ubiquinone reductase) was significantly lower in muscle mitochondria from patients with PD and MSA and a mean remaining activity < 30% of controls was observed. Mean activities of complexes III (ubiquinol:cytochrome c reductase) and IV (cytochrome c oxidase) were also lower in PD patients than controls, but a low activity (remaining activity < 30% of controls) was observed in only 5 PD patients for complex I and III or I and IV. No deficit in complex II activity (succinate:ubiquinone reductase) was observed. Our results support the hypothesis of a wide-spread mitochondrial complex I deficiency in PD and MSA as compared to age-matched controls, who showed age-related deficiency. This deficit can be found in de novo PD patients as well as in treated patients. The observed respiratory enzyme chain deficiency could not be explained by the dose and duration of L-Dopa or dopaminergic agonist treatment, the severity of the disease, anxiety or depression since no significant correlation was found between these parameters and enzyme complexes activities.</div>
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	La maladie de Parkinson en France (serveur d'exploration)
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La maladie de Parkinson en France (serveur d'exploration)

Mitochondrial respiratory failure in skeletal muscle from patients with Parkinson's disease and multiple system atrophy

Mitochondrial respiratory failure in skeletal muscle from patients with Parkinson's disease and multiple system atrophy

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