Mitochondrial Dysfunction in Cybrid Lines Expressing Mitochondrial Genes from Patients with Progressive Supranuclear Palsy
: Progressive supranuclear palsy (PSP) is a neurodegenerative movement disorder of unknown etiology. We hypothesized that mitochondrial DNA (mtDNA) aberration could occur in this disease and contribute to its pathogenesis. To address this we created transmitochondrial cytoplasmic hybrid (cybrid) cel...
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| Veröffentlicht in: | Journal of neurochemistry 2000-10, Vol.75 (4), p.1681-1684 |
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| creator | Swerdlow, R. H. Golbe, L. I. Parks, J. K. Cassarino, D. S. Binder, D. R. Grawey, A. E. Litvan, I. Bennett, J. P. Wooten, G. F. Parker, W. D. |
| description | : Progressive supranuclear palsy (PSP) is a
neurodegenerative movement disorder of unknown etiology. We hypothesized that
mitochondrial DNA (mtDNA) aberration could occur in this disease and
contribute to its pathogenesis. To address this we created transmitochondrial
cytoplasmic hybrid (cybrid) cell lines expressing mitochondrial genes from
persons with PSP. The presence of cybrid mtDNA aberration was screened for by
biochemical assay of mitochondrial gene products. Relative to a control cybrid
set, complex I activity was reduced in PSP cybrid lines (p < 0.005). Antioxidant enzyme activities were elevated in PSP cybrid lines. These data suggest that mtDNA aberration occurs in PSP, causes electron transport chain pathology, and can produce oxidative stress. Further study of mitochondrial dysfunction in PSP may yield insights into why neurodegeneration occurs in this disease. |
| doi_str_mv | 10.1046/j.1471-4159.2000.0751681.x |
| format | Article |
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neurodegenerative movement disorder of unknown etiology. We hypothesized that
mitochondrial DNA (mtDNA) aberration could occur in this disease and
contribute to its pathogenesis. To address this we created transmitochondrial
cytoplasmic hybrid (cybrid) cell lines expressing mitochondrial genes from
persons with PSP. The presence of cybrid mtDNA aberration was screened for by
biochemical assay of mitochondrial gene products. Relative to a control cybrid
set, complex I activity was reduced in PSP cybrid lines (p < 0.005). Antioxidant enzyme activities were elevated in PSP cybrid lines. These data suggest that mtDNA aberration occurs in PSP, causes electron transport chain pathology, and can produce oxidative stress. Further study of mitochondrial dysfunction in PSP may yield insights into why neurodegeneration occurs in this disease.</description><identifier>ISSN: 0022-3042</identifier><identifier>EISSN: 1471-4159</identifier><identifier>DOI: 10.1046/j.1471-4159.2000.0751681.x</identifier><identifier>PMID: 10987850</identifier><identifier>CODEN: JONRA9</identifier><language>eng</language><publisher>Oxford UK: Blackwell Science Ltd</publisher><subject>Aged ; Antioxidants - metabolism ; Biological and medical sciences ; Blood Platelets - cytology ; Catalase - metabolism ; Cell Fusion ; Cybrids ; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases ; DNA, Mitochondrial - genetics ; Electron Transport - genetics ; Electron Transport Complex I ; Electron Transport Complex IV - metabolism ; Female ; Glutathione Peroxidase - metabolism ; Glutathione Reductase - metabolism ; Humans ; Hybrid Cells - cytology ; Hybrid Cells - metabolism ; Male ; Medical sciences ; Mitochondria ; Mitochondria - enzymology ; Mitochondria - genetics ; NADH, NADPH Oxidoreductases - metabolism ; Neurology ; Oxidative stress ; Oxidative Stress - genetics ; Progressive supranuclear palsy ; Superoxide Dismutase - metabolism ; Supranuclear Palsy, Progressive - diagnosis ; Supranuclear Palsy, Progressive - etiology ; Supranuclear Palsy, Progressive - genetics ; Tumor Cells, Cultured</subject><ispartof>Journal of neurochemistry, 2000-10, Vol.75 (4), p.1681-1684</ispartof><rights>2001 INIST-CNRS</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c4851-cab099ee4fcceef0341d15f449d6ba43f1b2e8b39e9312a970970d385597c5bb3</citedby><cites>FETCH-LOGICAL-c4851-cab099ee4fcceef0341d15f449d6ba43f1b2e8b39e9312a970970d385597c5bb3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1046%2Fj.1471-4159.2000.0751681.x$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1046%2Fj.1471-4159.2000.0751681.x$$EHTML$$P50$$Gwiley$$H</linktohtml><link.rule.ids>314,776,780,1411,1427,27901,27902,45550,45551,46384,46808</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=826377$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/10987850$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Swerdlow, R. H.</creatorcontrib><creatorcontrib>Golbe, L. I.</creatorcontrib><creatorcontrib>Parks, J. K.</creatorcontrib><creatorcontrib>Cassarino, D. S.</creatorcontrib><creatorcontrib>Binder, D. R.</creatorcontrib><creatorcontrib>Grawey, A. E.</creatorcontrib><creatorcontrib>Litvan, I.</creatorcontrib><creatorcontrib>Bennett, J. P.</creatorcontrib><creatorcontrib>Wooten, G. F.</creatorcontrib><creatorcontrib>Parker, W. D.</creatorcontrib><title>Mitochondrial Dysfunction in Cybrid Lines Expressing Mitochondrial Genes from Patients with Progressive Supranuclear Palsy</title><title>Journal of neurochemistry</title><addtitle>J Neurochem</addtitle><description>: Progressive supranuclear palsy (PSP) is a
neurodegenerative movement disorder of unknown etiology. We hypothesized that
mitochondrial DNA (mtDNA) aberration could occur in this disease and
contribute to its pathogenesis. To address this we created transmitochondrial
cytoplasmic hybrid (cybrid) cell lines expressing mitochondrial genes from
persons with PSP. The presence of cybrid mtDNA aberration was screened for by
biochemical assay of mitochondrial gene products. Relative to a control cybrid
set, complex I activity was reduced in PSP cybrid lines (p < 0.005). Antioxidant enzyme activities were elevated in PSP cybrid lines. These data suggest that mtDNA aberration occurs in PSP, causes electron transport chain pathology, and can produce oxidative stress. Further study of mitochondrial dysfunction in PSP may yield insights into why neurodegeneration occurs in this disease.</description><subject>Aged</subject><subject>Antioxidants - metabolism</subject><subject>Biological and medical sciences</subject><subject>Blood Platelets - cytology</subject><subject>Catalase - metabolism</subject><subject>Cell Fusion</subject><subject>Cybrids</subject><subject>Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases</subject><subject>DNA, Mitochondrial - genetics</subject><subject>Electron Transport - genetics</subject><subject>Electron Transport Complex I</subject><subject>Electron Transport Complex IV - metabolism</subject><subject>Female</subject><subject>Glutathione Peroxidase - metabolism</subject><subject>Glutathione Reductase - metabolism</subject><subject>Humans</subject><subject>Hybrid Cells - cytology</subject><subject>Hybrid Cells - metabolism</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Mitochondria</subject><subject>Mitochondria - enzymology</subject><subject>Mitochondria - genetics</subject><subject>NADH, NADPH Oxidoreductases - metabolism</subject><subject>Neurology</subject><subject>Oxidative stress</subject><subject>Oxidative Stress - genetics</subject><subject>Progressive supranuclear palsy</subject><subject>Superoxide Dismutase - metabolism</subject><subject>Supranuclear Palsy, Progressive - diagnosis</subject><subject>Supranuclear Palsy, Progressive - etiology</subject><subject>Supranuclear Palsy, Progressive - genetics</subject><subject>Tumor Cells, Cultured</subject><issn>0022-3042</issn><issn>1471-4159</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2000</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqVkd1u1DAQRi0EotvCKyALJO4Sxj-JHe5gaQtogUrAteU4k9arbLK1E7rh6UnYqIIrhGRpZM35PCMfQp4zSBnI_NU2ZVKxRLKsSDkApKAylmuWHh6Q1X3rIVkBcJ4IkPyEnMa4BWC5zNljcsKg0EpnsCI_P_m-czddWwVvG_pujPXQut53LfUtXY9l8BXd-BYjPT_sA8bo22v6d-gS53Yduh29sr3Hto_0zvc39Cp0178jP5B-HfbBtoNr0IYJa-L4hDyqp4pPl3pGvl-cf1u_TzZfLj-s32wSJ3XGEmdLKApEWTuHWIOQrGJZLWVR5aWVomYlR12KAgvBuC0UTKcSOssK5bKyFGfk5fHdfehuB4y92fnosGlsi90QjeI806Dyf4JMKeBaywl8fQRd6GIMWJt98DsbRsPAzIrM1swezOzBzIrMosgcpvCzZcpQ7rD6I3p0MgEvFsBGZ5t6-jbn4z2neS6Umqi3R-rONzj-xwLm4-f1chG_APnsr8I</recordid><startdate>200010</startdate><enddate>200010</enddate><creator>Swerdlow, R. H.</creator><creator>Golbe, L. I.</creator><creator>Parks, J. K.</creator><creator>Cassarino, D. S.</creator><creator>Binder, D. R.</creator><creator>Grawey, A. E.</creator><creator>Litvan, I.</creator><creator>Bennett, J. P.</creator><creator>Wooten, G. F.</creator><creator>Parker, W. D.</creator><general>Blackwell Science Ltd</general><general>Blackwell</general><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7TK</scope><scope>7X8</scope></search><sort><creationdate>200010</creationdate><title>Mitochondrial Dysfunction in Cybrid Lines Expressing Mitochondrial Genes from Patients with Progressive Supranuclear Palsy</title><author>Swerdlow, R. H. ; Golbe, L. I. ; Parks, J. K. ; Cassarino, D. S. ; Binder, D. R. ; Grawey, A. E. ; Litvan, I. ; Bennett, J. P. ; Wooten, G. F. ; Parker, W. 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Prion diseases</topic><topic>DNA, Mitochondrial - genetics</topic><topic>Electron Transport - genetics</topic><topic>Electron Transport Complex I</topic><topic>Electron Transport Complex IV - metabolism</topic><topic>Female</topic><topic>Glutathione Peroxidase - metabolism</topic><topic>Glutathione Reductase - metabolism</topic><topic>Humans</topic><topic>Hybrid Cells - cytology</topic><topic>Hybrid Cells - metabolism</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Mitochondria</topic><topic>Mitochondria - enzymology</topic><topic>Mitochondria - genetics</topic><topic>NADH, NADPH Oxidoreductases - metabolism</topic><topic>Neurology</topic><topic>Oxidative stress</topic><topic>Oxidative Stress - genetics</topic><topic>Progressive supranuclear palsy</topic><topic>Superoxide Dismutase - metabolism</topic><topic>Supranuclear Palsy, Progressive - diagnosis</topic><topic>Supranuclear Palsy, Progressive - etiology</topic><topic>Supranuclear Palsy, Progressive - genetics</topic><topic>Tumor Cells, Cultured</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Swerdlow, R. H.</creatorcontrib><creatorcontrib>Golbe, L. I.</creatorcontrib><creatorcontrib>Parks, J. K.</creatorcontrib><creatorcontrib>Cassarino, D. S.</creatorcontrib><creatorcontrib>Binder, D. R.</creatorcontrib><creatorcontrib>Grawey, A. E.</creatorcontrib><creatorcontrib>Litvan, I.</creatorcontrib><creatorcontrib>Bennett, J. P.</creatorcontrib><creatorcontrib>Wooten, G. F.</creatorcontrib><creatorcontrib>Parker, W. D.</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Neurosciences Abstracts</collection><collection>MEDLINE - Academic</collection><jtitle>Journal of neurochemistry</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Swerdlow, R. H.</au><au>Golbe, L. I.</au><au>Parks, J. K.</au><au>Cassarino, D. S.</au><au>Binder, D. R.</au><au>Grawey, A. E.</au><au>Litvan, I.</au><au>Bennett, J. P.</au><au>Wooten, G. F.</au><au>Parker, W. D.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Mitochondrial Dysfunction in Cybrid Lines Expressing Mitochondrial Genes from Patients with Progressive Supranuclear Palsy</atitle><jtitle>Journal of neurochemistry</jtitle><addtitle>J Neurochem</addtitle><date>2000-10</date><risdate>2000</risdate><volume>75</volume><issue>4</issue><spage>1681</spage><epage>1684</epage><pages>1681-1684</pages><issn>0022-3042</issn><eissn>1471-4159</eissn><coden>JONRA9</coden><abstract>: Progressive supranuclear palsy (PSP) is a
neurodegenerative movement disorder of unknown etiology. We hypothesized that
mitochondrial DNA (mtDNA) aberration could occur in this disease and
contribute to its pathogenesis. To address this we created transmitochondrial
cytoplasmic hybrid (cybrid) cell lines expressing mitochondrial genes from
persons with PSP. The presence of cybrid mtDNA aberration was screened for by
biochemical assay of mitochondrial gene products. Relative to a control cybrid
set, complex I activity was reduced in PSP cybrid lines (p < 0.005). Antioxidant enzyme activities were elevated in PSP cybrid lines. These data suggest that mtDNA aberration occurs in PSP, causes electron transport chain pathology, and can produce oxidative stress. Further study of mitochondrial dysfunction in PSP may yield insights into why neurodegeneration occurs in this disease.</abstract><cop>Oxford UK</cop><pub>Blackwell Science Ltd</pub><pmid>10987850</pmid><doi>10.1046/j.1471-4159.2000.0751681.x</doi><tpages>4</tpages><oa>free_for_read</oa></addata></record> |
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| subjects | Aged Antioxidants - metabolism Biological and medical sciences Blood Platelets - cytology Catalase - metabolism Cell Fusion Cybrids Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases DNA, Mitochondrial - genetics Electron Transport - genetics Electron Transport Complex I Electron Transport Complex IV - metabolism Female Glutathione Peroxidase - metabolism Glutathione Reductase - metabolism Humans Hybrid Cells - cytology Hybrid Cells - metabolism Male Medical sciences Mitochondria Mitochondria - enzymology Mitochondria - genetics NADH, NADPH Oxidoreductases - metabolism Neurology Oxidative stress Oxidative Stress - genetics Progressive supranuclear palsy Superoxide Dismutase - metabolism Supranuclear Palsy, Progressive - diagnosis Supranuclear Palsy, Progressive - etiology Supranuclear Palsy, Progressive - genetics Tumor Cells, Cultured |
| title | Mitochondrial Dysfunction in Cybrid Lines Expressing Mitochondrial Genes from Patients with Progressive Supranuclear Palsy |
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