Mitochondrial respiratory failure in skeletal muscle from patients with Parkinson's disease and multiple system atrophy.
Identifieur interne : 001629 ( PubMed/Curation ); précédent : 001628; suivant : 001630Mitochondrial respiratory failure in skeletal muscle from patients with Parkinson's disease and multiple system atrophy.
Auteurs : O. Blin [France] ; C. Desnuelle ; O. Rascol ; M. Borg ; H. Peyro Saint Paul ; J P Azulay ; F. Billé ; D. Figarella ; F. Coulom ; J F PellissierSource :
- Journal of the neurological sciences [ 0022-510X ] ; 1994.
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), Histocytochemistry, Humans, Middle Aged, Mitochondria, Muscle (metabolism), Muscle, Skeletal (metabolism), NAD(P)H Dehydrogenase (Quinone) (metabolism), NADH, NADPH Oxidoreductases (metabolism), Nerve Degeneration, Oxygen Consumption, Parkinson Disease (metabolism), Regression Analysis.
- 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.
PubMed: 7964895
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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></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></titleStmt><publicationStmt><idno type="wicri:source">PubMed</idno><date when="1994">1994</date><idno type="RBID">pubmed:7964895</idno><idno type="pmid">7964895</idno><idno type="wicri:Area/PubMed/Corpus">001670</idno><idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Corpus" wicri:corpus="PubMed">001670</idno><idno type="wicri:Area/PubMed/Curation">001629</idno><idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Curation">001629</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en">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="1"><nlm:affiliation>Pharmacologie Médicale et Clinique, CHU Timone, Marseille, France.</nlm:affiliation><country xml:lang="fr">France</country><wicri:regionArea>Pharmacologie Médicale et Clinique, CHU Timone, Marseille</wicri:regionArea></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></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></analytic><series><title level="j">Journal of the neurological sciences</title><idno type="ISSN">0022-510X</idno><imprint><date when="1994" type="published">1994</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adult</term><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></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></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. 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><pubmed><MedlineCitation Status="MEDLINE" Owner="NLM"><PMID Version="1">7964895</PMID><DateCreated><Year>1994</Year><Month>12</Month><Day>20</Day></DateCreated><DateCompleted><Year>1994</Year><Month>12</Month><Day>20</Day></DateCompleted><DateRevised><Year>2007</Year><Month>11</Month><Day>15</Day></DateRevised><Article PubModel="Print"><Journal><ISSN IssnType="Print">0022-510X</ISSN><JournalIssue CitedMedium="Print"><Volume>125</Volume><Issue>1</Issue><PubDate><Year>1994</Year><Month>Aug</Month></PubDate></JournalIssue><Title>Journal of the neurological sciences</Title><ISOAbbreviation>J. Neurol. Sci.</ISOAbbreviation></Journal><ArticleTitle>Mitochondrial respiratory failure in skeletal muscle from patients with Parkinson's disease and multiple system atrophy.</ArticleTitle><Pagination><MedlinePgn>95-101</MedlinePgn></Pagination><Abstract><AbstractText>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.</AbstractText></Abstract><AuthorList CompleteYN="N"><Author ValidYN="Y"><LastName>Blin</LastName><ForeName>O</ForeName><Initials>O</Initials><AffiliationInfo><Affiliation>Pharmacologie Médicale et Clinique, CHU Timone, Marseille, France.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Desnuelle</LastName><ForeName>C</ForeName><Initials>C</Initials></Author><Author ValidYN="Y"><LastName>Rascol</LastName><ForeName>O</ForeName><Initials>O</Initials></Author><Author ValidYN="Y"><LastName>Borg</LastName><ForeName>M</ForeName><Initials>M</Initials></Author><Author ValidYN="Y"><LastName>Peyro Saint Paul</LastName><ForeName>H</ForeName><Initials>H</Initials></Author><Author ValidYN="Y"><LastName>Azulay</LastName><ForeName>J P</ForeName><Initials>JP</Initials></Author><Author ValidYN="Y"><LastName>Billé</LastName><ForeName>F</ForeName><Initials>F</Initials></Author><Author ValidYN="Y"><LastName>Figarella</LastName><ForeName>D</ForeName><Initials>D</Initials></Author><Author ValidYN="Y"><LastName>Coulom</LastName><ForeName>F</ForeName><Initials>F</Initials></Author><Author ValidYN="Y"><LastName>Pellissier</LastName><ForeName>J F</ForeName><Initials>JF</Initials></Author></AuthorList><Language>eng</Language><PublicationTypeList><PublicationType UI="D016428">Journal Article</PublicationType></PublicationTypeList></Article><MedlineJournalInfo><Country>Netherlands</Country><MedlineTA>J Neurol Sci</MedlineTA><NlmUniqueID>0375403</NlmUniqueID><ISSNLinking>0022-510X</ISSNLinking></MedlineJournalInfo><ChemicalList><Chemical><RegistryNumber>EC 1.10.2.2</RegistryNumber><NameOfSubstance UI="D014450">Electron Transport Complex III</NameOfSubstance></Chemical><Chemical><RegistryNumber>EC 1.6.-</RegistryNumber><NameOfSubstance UI="D009247">NADH, NADPH Oxidoreductases</NameOfSubstance></Chemical><Chemical><RegistryNumber>EC 1.6.5.2</RegistryNumber><NameOfSubstance UI="D016660">NAD(P)H Dehydrogenase (Quinone)</NameOfSubstance></Chemical><Chemical><RegistryNumber>EC 1.6.5.3</RegistryNumber><NameOfSubstance UI="D042967">Electron Transport Complex I</NameOfSubstance></Chemical><Chemical><RegistryNumber>EC 1.9.3.1</RegistryNumber><NameOfSubstance UI="D003576">Electron Transport Complex IV</NameOfSubstance></Chemical></ChemicalList><CitationSubset>IM</CitationSubset><MeshHeadingList><MeshHeading><DescriptorName UI="D000328" MajorTopicYN="N">Adult</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D000368" MajorTopicYN="N">Aged</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D000369" MajorTopicYN="N">Aged, 80 and over</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D001284" MajorTopicYN="N">Atrophy</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D001927" MajorTopicYN="N">Brain Diseases</DescriptorName><QualifierName UI="Q000378" MajorTopicYN="Y">metabolism</QualifierName><QualifierName UI="Q000473" MajorTopicYN="N">pathology</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D042967" MajorTopicYN="N">Electron Transport Complex I</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D014450" MajorTopicYN="N">Electron Transport Complex III</DescriptorName><QualifierName UI="Q000378" MajorTopicYN="N">metabolism</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D003576" MajorTopicYN="N">Electron Transport Complex IV</DescriptorName><QualifierName UI="Q000378" MajorTopicYN="N">metabolism</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D006651" MajorTopicYN="N">Histocytochemistry</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D006801" MajorTopicYN="N">Humans</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D008875" MajorTopicYN="N">Middle Aged</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D008931" MajorTopicYN="N">Mitochondria, Muscle</DescriptorName><QualifierName UI="Q000378" MajorTopicYN="Y">metabolism</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D018482" MajorTopicYN="N">Muscle, Skeletal</DescriptorName><QualifierName UI="Q000378" MajorTopicYN="Y">metabolism</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D016660" MajorTopicYN="N">NAD(P)H Dehydrogenase (Quinone)</DescriptorName><QualifierName UI="Q000378" MajorTopicYN="N">metabolism</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D009247" MajorTopicYN="N">NADH, NADPH Oxidoreductases</DescriptorName><QualifierName UI="Q000378" MajorTopicYN="N">metabolism</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D009410" MajorTopicYN="Y">Nerve Degeneration</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D010101" MajorTopicYN="Y">Oxygen Consumption</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D010300" MajorTopicYN="N">Parkinson Disease</DescriptorName><QualifierName UI="Q000378" MajorTopicYN="Y">metabolism</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D012044" MajorTopicYN="N">Regression Analysis</DescriptorName></MeshHeading></MeshHeadingList></MedlineCitation><PubmedData><History><PubMedPubDate PubStatus="pubmed"><Year>1994</Year><Month>8</Month><Day>1</Day></PubMedPubDate><PubMedPubDate PubStatus="medline"><Year>1994</Year><Month>8</Month><Day>1</Day><Hour>0</Hour><Minute>1</Minute></PubMedPubDate><PubMedPubDate PubStatus="entrez"><Year>1994</Year><Month>8</Month><Day>1</Day><Hour>0</Hour><Minute>0</Minute></PubMedPubDate></History><PublicationStatus>ppublish</PublicationStatus><ArticleIdList><ArticleId 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