Mitochondrial respiratory failure in skeletal muscle from patients with Parkinson's disease and multiple system atrophy
Identifieur interne : 001854 ( Istex/Corpus ); précédent : 001853; suivant : 001855Mitochondrial respiratory failure in skeletal muscle from patients with Parkinson's disease and multiple system atrophy
Auteurs : O. Blin ; C. Desnuelle ; O. Rascol ; M. Borg ; H. Peyro Saint Paul ; J. P. Azulay ; F. Billé ; D. Figarella ; F. Coulom ; J. F. Pellissier ; J. L. Montastruc ; M. Chatel ; G. SerratriceSource :
- Journal of the Neurological Sciences [ 0022-510X ] ; 1994.
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.
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DOI: 10.1016/0022-510X(94)90248-8
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Rascol</name><affiliation><mods:affiliation>Laboratoire de Pharmacologie Médicale et Clinique, Faculté de Médecine, 31073 Toulouse, France</mods:affiliation></affiliation></author><author><name sortKey="Borg, M" sort="Borg, M" uniqKey="Borg M" first="M." last="Borg">M. Borg</name><affiliation><mods:affiliation>Service de Neurologie, CHU Pasteur, 06000 Nice, France</mods:affiliation></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><mods:affiliation>Laboratoire de Pharmacologie Médicale et Clinique, Faculté de Médecine, 31073 Toulouse, France</mods:affiliation></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><mods:affiliation>Clinique des Maladies du Système nerveux et de l'Appareil Locomoteur, CHU Timone, 13385 Marseille Cedex 5, France</mods:affiliation></affiliation></author><author><name sortKey="Bille, F" sort="Bille, F" uniqKey="Bille F" first="F." last="Billé">F. Billé</name><affiliation><mods:affiliation>Clinique des Maladies du Système nerveux et de l'Appareil Locomoteur, CHU Timone, 13385 Marseille Cedex 5, France</mods:affiliation></affiliation></author><author><name sortKey="Figarella, D" sort="Figarella, D" uniqKey="Figarella D" first="D." last="Figarella">D. Figarella</name><affiliation><mods:affiliation>Département de Neuropathologie, CHU Timone, 13385 Marseille Cedex 5, France</mods:affiliation></affiliation></author><author><name sortKey="Coulom, F" sort="Coulom, F" uniqKey="Coulom F" first="F." last="Coulom">F. 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Serratrice</name><affiliation><mods:affiliation>Clinique des Maladies du Système nerveux et de l'Appareil Locomoteur, CHU Timone, 13385 Marseille Cedex 5, France</mods:affiliation></affiliation></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><idno type="url">https://api.istex.fr/document/0369D0033894298A8324FB758ADC53C38CBF1146/fulltext/pdf</idno><idno type="wicri:Area/Istex/Corpus">001854</idno><idno type="wicri:explorRef" wicri:stream="Istex" wicri:step="Corpus" wicri:corpus="ISTEX">001854</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><mods:affiliation>Pharmacologie Médicale et Clinique and CPCET, CHU Timone, 13385 Marseille Cedex 5, France</mods:affiliation></affiliation></author><author><name sortKey="Desnuelle, C" sort="Desnuelle, C" uniqKey="Desnuelle C" first="C." last="Desnuelle">C. Desnuelle</name><affiliation><mods:affiliation>Service de Neurologie, CHU Pasteur, 06000 Nice, France</mods:affiliation></affiliation></author><author><name sortKey="Rascol, O" sort="Rascol, O" uniqKey="Rascol O" first="O." last="Rascol">O. Rascol</name><affiliation><mods:affiliation>Laboratoire de Pharmacologie Médicale et Clinique, Faculté de Médecine, 31073 Toulouse, France</mods:affiliation></affiliation></author><author><name sortKey="Borg, M" sort="Borg, M" uniqKey="Borg M" first="M." last="Borg">M. Borg</name><affiliation><mods:affiliation>Service de Neurologie, CHU Pasteur, 06000 Nice, France</mods:affiliation></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><mods:affiliation>Laboratoire de Pharmacologie Médicale et Clinique, Faculté de Médecine, 31073 Toulouse, France</mods:affiliation></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><mods:affiliation>Clinique des Maladies du Système nerveux et de l'Appareil Locomoteur, CHU Timone, 13385 Marseille Cedex 5, France</mods:affiliation></affiliation></author><author><name sortKey="Bille, F" sort="Bille, F" uniqKey="Bille F" first="F." last="Billé">F. Billé</name><affiliation><mods:affiliation>Clinique des Maladies du Système nerveux et de l'Appareil Locomoteur, CHU Timone, 13385 Marseille Cedex 5, France</mods:affiliation></affiliation></author><author><name sortKey="Figarella, D" sort="Figarella, D" uniqKey="Figarella D" first="D." last="Figarella">D. Figarella</name><affiliation><mods:affiliation>Département de Neuropathologie, CHU Timone, 13385 Marseille Cedex 5, France</mods:affiliation></affiliation></author><author><name sortKey="Coulom, F" sort="Coulom, F" uniqKey="Coulom F" first="F." last="Coulom">F. Coulom</name><affiliation><mods:affiliation>Clinique des Maladies du Système nerveux et de l'Appareil Locomoteur, CHU Timone, 13385 Marseille Cedex 5, France</mods:affiliation></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><mods:affiliation>Département de Neuropathologie, CHU Timone, 13385 Marseille Cedex 5, France</mods:affiliation></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><mods:affiliation>Laboratoire de Pharmacologie Médicale et Clinique, Faculté de Médecine, 31073 Toulouse, France</mods:affiliation></affiliation></author><author><name sortKey="Chatel, M" sort="Chatel, M" uniqKey="Chatel M" first="M." last="Chatel">M. Chatel</name><affiliation><mods:affiliation>Service de Neurologie, CHU Pasteur, 06000 Nice, France</mods:affiliation></affiliation></author><author><name sortKey="Serratrice, G" sort="Serratrice, G" uniqKey="Serratrice G" first="G." last="Serratrice">G. Serratrice</name><affiliation><mods:affiliation>Clinique des Maladies du Système nerveux et de l'Appareil Locomoteur, CHU Timone, 13385 Marseille Cedex 5, France</mods:affiliation></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><biblScope unit="page" to="101">101</biblScope></imprint><idno type="ISSN">0022-510X</idno></series><idno type="istex">0369D0033894298A8324FB758ADC53C38CBF1146</idno><idno type="DOI">10.1016/0022-510X(94)90248-8</idno><idno type="PII">0022-510X(94)90248-8</idno></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0022-510X</idno></seriesStmt></fileDesc><profileDesc><textClass></textClass><langUsage><language ident="en">en</language></langUsage></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. 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></front></TEI><istex><corpusName>elsevier</corpusName><author><json:item><name>O. Blin</name><affiliations><json:string>Pharmacologie Médicale et Clinique and CPCET, CHU Timone, 13385 Marseille Cedex 5, France</json:string></affiliations></json:item><json:item><name>C. Desnuelle</name><affiliations><json:string>Service de Neurologie, CHU Pasteur, 06000 Nice, France</json:string></affiliations></json:item><json:item><name>O. Rascol</name><affiliations><json:string>Laboratoire de Pharmacologie Médicale et Clinique, Faculté de Médecine, 31073 Toulouse, France</json:string></affiliations></json:item><json:item><name>M. Borg</name><affiliations><json:string>Service de Neurologie, CHU Pasteur, 06000 Nice, France</json:string></affiliations></json:item><json:item><name>H.Peyro Saint Paul</name><affiliations><json:string>Laboratoire de Pharmacologie Médicale et Clinique, Faculté de Médecine, 31073 Toulouse, France</json:string></affiliations></json:item><json:item><name>J.P. Azulay</name><affiliations><json:string>Clinique des Maladies du Système nerveux et de l'Appareil Locomoteur, CHU Timone, 13385 Marseille Cedex 5, France</json:string></affiliations></json:item><json:item><name>F. Billé</name><affiliations><json:string>Clinique des Maladies du Système nerveux et de l'Appareil Locomoteur, CHU Timone, 13385 Marseille Cedex 5, France</json:string></affiliations></json:item><json:item><name>D. Figarella</name><affiliations><json:string>Département de Neuropathologie, CHU Timone, 13385 Marseille Cedex 5, France</json:string></affiliations></json:item><json:item><name>F. Coulom</name><affiliations><json:string>Clinique des Maladies du Système nerveux et de l'Appareil Locomoteur, CHU Timone, 13385 Marseille Cedex 5, France</json:string></affiliations></json:item><json:item><name>J.F. Pellissier</name><affiliations><json:string>Département de Neuropathologie, CHU Timone, 13385 Marseille Cedex 5, France</json:string></affiliations></json:item><json:item><name>J.L. Montastruc</name><affiliations><json:string>Laboratoire de Pharmacologie Médicale et Clinique, Faculté de Médecine, 31073 Toulouse, France</json:string></affiliations></json:item><json:item><name>M. Chatel</name><affiliations><json:string>Service de Neurologie, CHU Pasteur, 06000 Nice, France</json:string></affiliations></json:item><json:item><name>G. Serratrice</name><affiliations><json:string>Clinique des Maladies du Système nerveux et de l'Appareil Locomoteur, CHU Timone, 13385 Marseille Cedex 5, France</json:string></affiliations></json:item></author><subject><json:item><lang><json:string>eng</json:string></lang><value>Clinical section</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>Parkinson's disease</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>Mitochondrial respiratory chain</value></json:item></subject><language><json:string>eng</json:string></language><originalGenre><json:string>Full-length article</json:string></originalGenre><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.</abstract><qualityIndicators><score>7.415</score><pdfVersion>1.2</pdfVersion><pdfPageSize>612 x 828 pts</pdfPageSize><refBibsNative>true</refBibsNative><keywordCount>3</keywordCount><abstractCharCount>1641</abstractCharCount><pdfWordCount>4439</pdfWordCount><pdfCharCount>27389</pdfCharCount><pdfPageCount>7</pdfPageCount><abstractWordCount>248</abstractWordCount></qualityIndicators><title>Mitochondrial respiratory failure in skeletal muscle from patients with Parkinson's disease and multiple system atrophy</title><pii><json:string>0022-510X(94)90248-8</json:string></pii><refBibs><json:item><author><json:item><name>BAC Ackrell</name></json:item><json:item><name>EB Kearney</name></json:item><json:item><name>TP Singer</name></json:item></author><host><volume>53</volume><pages><last>483</last><first>466</first></pages><author></author><title>Methods Enzymol</title></host><title>Mammalian succinate deshydrogenase</title></json:item><json:item><author><json:item><name>JJ Anderson</name></json:item><json:item><name>D Bravi</name></json:item><json:item><name>R Ferrari</name></json:item><json:item><name>TL Davis</name></json:item><json:item><name>F Baronti</name></json:item><json:item><name>TN Chase</name></json:item><json:item><name>F Dagani</name></json:item></author><host><volume>56</volume><pages><last>480</last><first>477</first></pages><author></author><title>J Neurol Neurosurg Psychiat</title></host><title>No evidence for altered muscle mitochondrial function in Parkinson's disease</title></json:item><json:item><author><json:item><name>G Bellomo</name></json:item><json:item><name>SA Jewell</name></json:item><json:item><name>S Orrenius</name></json:item></author><host><volume>257</volume><pages><last>1162</last><first>1158</first></pages><author></author><title>J Biol Chem</title></host><title>The metabolism of menadione impairs the ability of rat liver mitochondria to take up and retain calcium</title></json:item><json:item><author><json:item><name>LA Bindoff</name></json:item><json:item><name>M Birch-Machin</name></json:item><json:item><name>NEF Cartlidge</name></json:item><json:item><name>WD Parker, Jr</name></json:item><json:item><name>DM Turnbull</name></json:item></author><host><volume>i</volume><pages><first>49</first></pages><author></author><title>Lancet</title></host><title>Mitochondrial function in Parkinson's disease</title></json:item><json:item><author><json:item><name>LA Bindoff</name></json:item><json:item><name>C Desnuelle</name></json:item><json:item><name>MA Birch-Machin</name></json:item><json:item><name>JF Pellissier</name></json:item><json:item><name>G Serratrice</name></json:item><json:item><name>C Dravet</name></json:item><json:item><name>M Bureau</name></json:item><json:item><name>N Howell</name></json:item><json:item><name>DM Turnbull</name></json:item></author><host><volume>102</volume><pages><last>24</last><first>17</first></pages><author></author><title>J Neurol Sci</title></host><title>Multiple defects of the mitochondrial respiratory chain in a mitochondrial encephalopathy (MERRF): a clinical, biochemical and molecular study</title></json:item><json:item><author><json:item><name>LA Bindoff</name></json:item><json:item><name>M Birch-Machin</name></json:item><json:item><name>NEF Cartlidge</name></json:item><json:item><name>WD Parker, Jr</name></json:item><json:item><name>DM Turnbull</name></json:item></author><host><volume>104</volume><pages><last>208</last><first>203</first></pages><author></author><title>J Neurol Sci</title></host><title>Respiratory chain abnormalities in skeletal muscle from patients with Parkinson's disease</title></json:item><json:item><author><json:item><name>D Bravi</name></json:item><json:item><name>JA Jeffrey</name></json:item><json:item><name>F Dagani</name></json:item><json:item><name>TL Davis</name></json:item><json:item><name>R Ferrari</name></json:item><json:item><name>M. 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Sciences</title><publicationDate>1994</publicationDate></host><categories><wos><json:string>science</json:string><json:string>neurosciences</json:string><json:string>clinical neurology</json:string></wos><scienceMetrix></scienceMetrix></categories><publicationDate>1994</publicationDate><copyrightDate>1994</copyrightDate><doi><json:string>10.1016/0022-510X(94)90248-8</json:string></doi><id>0369D0033894298A8324FB758ADC53C38CBF1146</id><score>0.16877982</score><fulltext><json:item><extension>pdf</extension><original>true</original><mimetype>application/pdf</mimetype><uri>https://api.istex.fr/document/0369D0033894298A8324FB758ADC53C38CBF1146/fulltext/pdf</uri></json:item><json:item><extension>zip</extension><original>false</original><mimetype>application/zip</mimetype><uri>https://api.istex.fr/document/0369D0033894298A8324FB758ADC53C38CBF1146/fulltext/zip</uri></json:item><istex:fulltextTEI uri="https://api.istex.fr/document/0369D0033894298A8324FB758ADC53C38CBF1146/fulltext/tei"><teiHeader><fileDesc><titleStmt><title level="a">Mitochondrial respiratory failure in skeletal muscle from patients with Parkinson's disease and multiple system atrophy</title></titleStmt><publicationStmt><authority>ISTEX</authority><publisher>ELSEVIER</publisher><availability><p>ELSEVIER</p></availability><date>1994</date></publicationStmt><notesStmt><note type="content">Section title: Research article</note></notesStmt><sourceDesc><biblStruct type="inbook"><analytic><title level="a">Mitochondrial respiratory failure in skeletal muscle from patients with Parkinson's disease and multiple system atrophy</title><author xml:id="author-1"><persName><forename type="first">O.</forename><surname>Blin</surname></persName><note type="correspondence"><p>Corresponding author.</p></note><affiliation>Pharmacologie Médicale et Clinique and CPCET, CHU Timone, 13385 Marseille Cedex 5, France</affiliation></author><author xml:id="author-2"><persName><forename type="first">C.</forename><surname>Desnuelle</surname></persName><affiliation>Service de Neurologie, CHU Pasteur, 06000 Nice, France</affiliation></author><author xml:id="author-3"><persName><forename type="first">O.</forename><surname>Rascol</surname></persName><affiliation>Laboratoire de Pharmacologie Médicale et Clinique, Faculté de Médecine, 31073 Toulouse, France</affiliation></author><author xml:id="author-4"><persName><forename type="first">M.</forename><surname>Borg</surname></persName><affiliation>Service de Neurologie, CHU Pasteur, 06000 Nice, France</affiliation></author><author xml:id="author-5"><persName><forename type="first">H.Peyro Saint</forename><surname>Paul</surname></persName><affiliation>Laboratoire de Pharmacologie Médicale et Clinique, Faculté de Médecine, 31073 Toulouse, France</affiliation></author><author xml:id="author-6"><persName><forename type="first">J.P.</forename><surname>Azulay</surname></persName><affiliation>Clinique des Maladies du Système nerveux et de l'Appareil Locomoteur, CHU Timone, 13385 Marseille Cedex 5, France</affiliation></author><author xml:id="author-7"><persName><forename type="first">F.</forename><surname>Billé</surname></persName><affiliation>Clinique des Maladies du Système nerveux et de l'Appareil Locomoteur, CHU Timone, 13385 Marseille Cedex 5, France</affiliation></author><author xml:id="author-8"><persName><forename type="first">D.</forename><surname>Figarella</surname></persName><affiliation>Département de Neuropathologie, CHU Timone, 13385 Marseille Cedex 5, France</affiliation></author><author xml:id="author-9"><persName><forename type="first">F.</forename><surname>Coulom</surname></persName><affiliation>Clinique des Maladies du Système nerveux et de l'Appareil Locomoteur, CHU Timone, 13385 Marseille Cedex 5, France</affiliation></author><author xml:id="author-10"><persName><forename type="first">J.F.</forename><surname>Pellissier</surname></persName><affiliation>Département de Neuropathologie, CHU Timone, 13385 Marseille Cedex 5, France</affiliation></author><author xml:id="author-11"><persName><forename type="first">J.L.</forename><surname>Montastruc</surname></persName><affiliation>Laboratoire de Pharmacologie Médicale et Clinique, Faculté de Médecine, 31073 Toulouse, France</affiliation></author><author xml:id="author-12"><persName><forename type="first">M.</forename><surname>Chatel</surname></persName><affiliation>Service de Neurologie, CHU Pasteur, 06000 Nice, France</affiliation></author><author xml:id="author-13"><persName><forename type="first">G.</forename><surname>Serratrice</surname></persName><affiliation>Clinique des Maladies du Système nerveux et de l'Appareil Locomoteur, CHU Timone, 13385 Marseille Cedex 5, France</affiliation></author></analytic><monogr><title level="j">Journal of the Neurological Sciences</title><title level="j" type="abbrev">JNS</title><idno type="pISSN">0022-510X</idno><idno type="PII">S0022-510X(00)X0395-4</idno><imprint><publisher>ELSEVIER</publisher><date type="published" when="1994"></date><biblScope unit="volume">125</biblScope><biblScope unit="issue">1</biblScope><biblScope unit="page" from="95">95</biblScope><biblScope unit="page" to="101">101</biblScope></imprint></monogr><idno type="istex">0369D0033894298A8324FB758ADC53C38CBF1146</idno><idno type="DOI">10.1016/0022-510X(94)90248-8</idno><idno type="PII">0022-510X(94)90248-8</idno></biblStruct></sourceDesc></fileDesc><profileDesc><creation><date>1994</date></creation><langUsage><language ident="en">en</language></langUsage><abstract xml:lang="en"><p>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.</p></abstract><textClass><keywords scheme="keyword"><list><head>article-category</head><item><term>Clinical section</term></item></list></keywords></textClass><textClass><keywords scheme="keyword"><list><head>Keywords</head><item><term>Parkinson's disease</term></item><item><term>Mitochondrial respiratory chain</term></item></list></keywords></textClass></profileDesc><revisionDesc><change when="1994-03-15">Modified</change><change when="1994">Published</change></revisionDesc></teiHeader></istex:fulltextTEI><json:item><extension>txt</extension><original>false</original><mimetype>text/plain</mimetype><uri>https://api.istex.fr/document/0369D0033894298A8324FB758ADC53C38CBF1146/fulltext/txt</uri></json:item></fulltext><metadata><istex:metadataXml wicri:clean="Elsevier, elements deleted: tail"><istex:xmlDeclaration>version="1.0" encoding="utf-8"</istex:xmlDeclaration><istex:docType PUBLIC="-//ES//DTD journal article DTD version 4.5.2//EN//XML" URI="art452.dtd" name="istex:docType"></istex:docType><istex:document><converted-article version="4.5.2" docsubtype="fla"><item-info><jid>JNS</jid><aid>94902488</aid><ce:pii>0022-510X(94)90248-8</ce:pii><ce:doi>10.1016/0022-510X(94)90248-8</ce:doi><ce:copyright type="unknown" year="1994"></ce:copyright><ce:doctopics><ce:doctopic><ce:text>Clinical section</ce:text></ce:doctopic></ce:doctopics></item-info><head><ce:dochead><ce:textfn>Research article</ce:textfn></ce:dochead><ce:title>Mitochondrial respiratory failure in skeletal muscle from patients with Parkinson's disease and multiple system atrophy</ce:title><ce:author-group><ce:author><ce:given-name>O.</ce:given-name><ce:surname>Blin</ce:surname><ce:cross-ref refid="COR1"><ce:sup>∗</ce:sup></ce:cross-ref><ce:cross-ref refid="AFF1"><ce:sup>a</ce:sup></ce:cross-ref></ce:author><ce:author><ce:given-name>C.</ce:given-name><ce:surname>Desnuelle</ce:surname><ce:cross-ref refid="AFF4"><ce:sup>d</ce:sup></ce:cross-ref></ce:author><ce:author><ce:given-name>O.</ce:given-name><ce:surname>Rascol</ce:surname><ce:cross-ref refid="AFF5"><ce:sup>e</ce:sup></ce:cross-ref></ce:author><ce:author><ce:given-name>M.</ce:given-name><ce:surname>Borg</ce:surname><ce:cross-ref refid="AFF4"><ce:sup>d</ce:sup></ce:cross-ref></ce:author><ce:author><ce:given-name>H.Peyro Saint</ce:given-name><ce:surname>Paul</ce:surname><ce:cross-ref refid="AFF5"><ce:sup>e</ce:sup></ce:cross-ref></ce:author><ce:author><ce:given-name>J.P.</ce:given-name><ce:surname>Azulay</ce:surname><ce:cross-ref refid="AFF2"><ce:sup>b</ce:sup></ce:cross-ref></ce:author><ce:author><ce:given-name>F.</ce:given-name><ce:surname>Billé</ce:surname><ce:cross-ref refid="AFF2"><ce:sup>b</ce:sup></ce:cross-ref></ce:author><ce:author><ce:given-name>D.</ce:given-name><ce:surname>Figarella</ce:surname><ce:cross-ref refid="AFF3"><ce:sup>c</ce:sup></ce:cross-ref></ce:author><ce:author><ce:given-name>F.</ce:given-name><ce:surname>Coulom</ce:surname><ce:cross-ref refid="AFF2"><ce:sup>b</ce:sup></ce:cross-ref></ce:author><ce:author><ce:given-name>J.F.</ce:given-name><ce:surname>Pellissier</ce:surname><ce:cross-ref refid="AFF3"><ce:sup>c</ce:sup></ce:cross-ref></ce:author><ce:author><ce:given-name>J.L.</ce:given-name><ce:surname>Montastruc</ce:surname><ce:cross-ref refid="AFF5"><ce:sup>e</ce:sup></ce:cross-ref></ce:author><ce:author><ce:given-name>M.</ce:given-name><ce:surname>Chatel</ce:surname><ce:cross-ref refid="AFF4"><ce:sup>d</ce:sup></ce:cross-ref></ce:author><ce:author><ce:given-name>G.</ce:given-name><ce:surname>Serratrice</ce:surname><ce:cross-ref refid="AFF2"><ce:sup>b</ce:sup></ce:cross-ref></ce:author><ce:affiliation id="AFF1"><ce:label>a</ce:label><ce:textfn>Pharmacologie Médicale et Clinique and CPCET, CHU Timone, 13385 Marseille Cedex 5, France</ce:textfn></ce:affiliation><ce:affiliation id="AFF2"><ce:label>b</ce:label><ce:textfn>Clinique des Maladies du Système nerveux et de l'Appareil Locomoteur, CHU Timone, 13385 Marseille Cedex 5, France</ce:textfn></ce:affiliation><ce:affiliation id="AFF3"><ce:label>c</ce:label><ce:textfn>Département de Neuropathologie, CHU Timone, 13385 Marseille Cedex 5, France</ce:textfn></ce:affiliation><ce:affiliation id="AFF4"><ce:label>d</ce:label><ce:textfn>Service de Neurologie, CHU Pasteur, 06000 Nice, France</ce:textfn></ce:affiliation><ce:affiliation id="AFF5"><ce:label>e</ce:label><ce:textfn>Laboratoire de Pharmacologie Médicale et Clinique, Faculté de Médecine, 31073 Toulouse, France</ce:textfn></ce:affiliation><ce:correspondence id="COR1"><ce:label>∗</ce:label><ce:text>Corresponding author.</ce:text></ce:correspondence></ce:author-group><ce:date-received day="31" month="8" year="1993"></ce:date-received><ce:date-revised day="15" month="3" year="1994"></ce:date-revised><ce:date-accepted day="29" month="3" year="1994"></ce:date-accepted><ce:abstract><ce:section-title>Abstract</ce:section-title><ce:abstract-sec><ce:simple-para>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 <ce:italic>c</ce:italic> reductase) and IV (cytochrome <ce:italic>c</ce:italic> 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 <ce:small-caps>l</ce:small-caps>-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.</ce:simple-para></ce:abstract-sec></ce:abstract><ce:keywords><ce:section-title>Keywords</ce:section-title><ce:keyword><ce:text>Parkinson's disease</ce:text></ce:keyword><ce:keyword><ce:text>Mitochondrial respiratory chain</ce:text></ce:keyword></ce:keywords></head></converted-article></istex:document></istex:metadataXml><mods version="3.6"><titleInfo><title>Mitochondrial respiratory failure in skeletal muscle from patients with Parkinson's disease and multiple system atrophy</title></titleInfo><titleInfo type="alternative" contentType="CDATA"><title>Mitochondrial respiratory failure in skeletal muscle from patients with Parkinson's disease and multiple system atrophy</title></titleInfo><name type="personal"><namePart type="given">O.</namePart><namePart type="family">Blin</namePart><affiliation>Pharmacologie Médicale et Clinique and CPCET, CHU Timone, 13385 Marseille Cedex 5, France</affiliation><description>Corresponding author.</description><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">C.</namePart><namePart type="family">Desnuelle</namePart><affiliation>Service de Neurologie, CHU Pasteur, 06000 Nice, France</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">O.</namePart><namePart type="family">Rascol</namePart><affiliation>Laboratoire de Pharmacologie Médicale et Clinique, Faculté de Médecine, 31073 Toulouse, France</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">M.</namePart><namePart type="family">Borg</namePart><affiliation>Service de Neurologie, CHU Pasteur, 06000 Nice, France</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">H.Peyro Saint</namePart><namePart type="family">Paul</namePart><affiliation>Laboratoire de Pharmacologie Médicale et Clinique, Faculté de Médecine, 31073 Toulouse, France</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">J.P.</namePart><namePart type="family">Azulay</namePart><affiliation>Clinique des Maladies du Système nerveux et de l'Appareil Locomoteur, CHU Timone, 13385 Marseille Cedex 5, France</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">F.</namePart><namePart type="family">Billé</namePart><affiliation>Clinique des Maladies du Système nerveux et de l'Appareil Locomoteur, CHU Timone, 13385 Marseille Cedex 5, France</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">D.</namePart><namePart type="family">Figarella</namePart><affiliation>Département de Neuropathologie, CHU Timone, 13385 Marseille Cedex 5, France</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">F.</namePart><namePart type="family">Coulom</namePart><affiliation>Clinique des Maladies du Système nerveux et de l'Appareil Locomoteur, CHU Timone, 13385 Marseille Cedex 5, France</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">J.F.</namePart><namePart type="family">Pellissier</namePart><affiliation>Département de Neuropathologie, CHU Timone, 13385 Marseille Cedex 5, France</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">J.L.</namePart><namePart type="family">Montastruc</namePart><affiliation>Laboratoire de Pharmacologie Médicale et Clinique, Faculté de Médecine, 31073 Toulouse, France</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">M.</namePart><namePart type="family">Chatel</namePart><affiliation>Service de Neurologie, CHU Pasteur, 06000 Nice, France</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">G.</namePart><namePart type="family">Serratrice</namePart><affiliation>Clinique des Maladies du Système nerveux et de l'Appareil Locomoteur, CHU Timone, 13385 Marseille Cedex 5, France</affiliation><role><roleTerm type="text">author</roleTerm></role></name><typeOfResource>text</typeOfResource><genre type="research-article" displayLabel="Full-length article"></genre><originInfo><publisher>ELSEVIER</publisher><dateIssued encoding="w3cdtf">1994</dateIssued><dateModified encoding="w3cdtf">1994-03-15</dateModified><copyrightDate encoding="w3cdtf">1994</copyrightDate></originInfo><language><languageTerm type="code" authority="iso639-2b">eng</languageTerm><languageTerm type="code" authority="rfc3066">en</languageTerm></language><physicalDescription><internetMediaType>text/html</internetMediaType></physicalDescription><abstract 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.</abstract><note type="content">Section title: Research article</note><subject><genre>article-category</genre><topic>Clinical section</topic></subject><subject><genre>Keywords</genre><topic>Parkinson's disease</topic><topic>Mitochondrial respiratory chain</topic></subject><relatedItem type="host"><titleInfo><title>Journal of the Neurological Sciences</title></titleInfo><titleInfo type="abbreviated"><title>JNS</title></titleInfo><genre type="journal">journal</genre><originInfo><dateIssued encoding="w3cdtf">199408</dateIssued></originInfo><identifier type="ISSN">0022-510X</identifier><identifier type="PII">S0022-510X(00)X0395-4</identifier><part><date>199408</date><detail type="volume"><number>125</number><caption>vol.</caption></detail><detail type="issue"><number>1</number><caption>no.</caption></detail><extent unit="issue pages"><start>1</start><end>118</end></extent><extent unit="pages"><start>95</start><end>101</end></extent></part></relatedItem><identifier type="istex">0369D0033894298A8324FB758ADC53C38CBF1146</identifier><identifier type="DOI">10.1016/0022-510X(94)90248-8</identifier><identifier type="PII">0022-510X(94)90248-8</identifier><recordInfo><recordContentSource>ELSEVIER</recordContentSource></recordInfo></mods></metadata><serie></serie></istex></record>Pour manipuler ce document sous Unix (Dilib)
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