Further Evidence for Mitochondrial Dysfunction in Progressive Supranuclear Palsy
Recent data from our laboratory have identified a role for mitochondrial dysfunction in the pathogenesis of progressive supranuclear palsy (PSP). To extend this finding, we measured key parameters of mitochondrial function in platelet-derived cytoplasmic hybrid (cybrid) cell lines expressing mitocho...
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| Veröffentlicht in: | Experimental neurology 2001-03, Vol.168 (1), p.196-198 |
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| container_end_page | 198 |
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| container_issue | 1 |
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| container_title | Experimental neurology |
| container_volume | 168 |
| creator | Albers, David S. Swerdlow, Russell H. Manfredi, Giovanni Gajewski, Carl Yang, Lichuan Parker, W.Davis Beal, M.Flint |
| description | Recent data from our laboratory have identified a role for mitochondrial dysfunction in the pathogenesis of progressive supranuclear palsy (PSP). To extend this finding, we measured key parameters of mitochondrial function in platelet-derived cytoplasmic hybrid (cybrid) cell lines expressing mitochondrial genes from patients with PSP. We observed significant decreases in aconitase activity, cellular ATP levels, and oxygen consumption in PSP cybrids as compared to control cybrids, further suggesting a contributory role of impaired mitochondrial energy metabolism in PSP, possibly due to genetic abnormalities of mitochondrial DNA. |
| doi_str_mv | 10.1006/exnr.2000.7607 |
| format | Article |
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To extend this finding, we measured key parameters of mitochondrial function in platelet-derived cytoplasmic hybrid (cybrid) cell lines expressing mitochondrial genes from patients with PSP. We observed significant decreases in aconitase activity, cellular ATP levels, and oxygen consumption in PSP cybrids as compared to control cybrids, further suggesting a contributory role of impaired mitochondrial energy metabolism in PSP, possibly due to genetic abnormalities of mitochondrial DNA.</description><identifier>ISSN: 0014-4886</identifier><identifier>EISSN: 1090-2430</identifier><identifier>DOI: 10.1006/exnr.2000.7607</identifier><identifier>PMID: 11170735</identifier><identifier>CODEN: EXNEAC</identifier><language>eng</language><publisher>Amsterdam: Elsevier Inc</publisher><subject>aconitase ; Adenosine Triphosphate - metabolism ; Aged ; ATP ; Biological and medical sciences ; Blood Platelets - pathology ; Blood Platelets - physiology ; Cell Fusion ; cybrids ; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. 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To extend this finding, we measured key parameters of mitochondrial function in platelet-derived cytoplasmic hybrid (cybrid) cell lines expressing mitochondrial genes from patients with PSP. We observed significant decreases in aconitase activity, cellular ATP levels, and oxygen consumption in PSP cybrids as compared to control cybrids, further suggesting a contributory role of impaired mitochondrial energy metabolism in PSP, possibly due to genetic abnormalities of mitochondrial DNA.</description><subject>aconitase</subject><subject>Adenosine Triphosphate - metabolism</subject><subject>Aged</subject><subject>ATP</subject><subject>Biological and medical sciences</subject><subject>Blood Platelets - pathology</subject><subject>Blood Platelets - physiology</subject><subject>Cell Fusion</subject><subject>cybrids</subject><subject>Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases</subject><subject>Female</subject><subject>Humans</subject><subject>Hybrid Cells</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Mitochondria - metabolism</subject><subject>Neuroblastoma</subject><subject>neurodegeneration</subject><subject>Neurology</subject><subject>oxygen</subject><subject>Oxygen Consumption</subject><subject>Reference Values</subject><subject>Supranuclear Palsy, Progressive - blood</subject><subject>Supranuclear Palsy, Progressive - metabolism</subject><subject>Tumor Cells, Cultured</subject><issn>0014-4886</issn><issn>1090-2430</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2001</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp10M9r2zAUwHExOpa023XHYijs5uzphyX7WNq0HXQssO0sFOmpUXGsVLJD89_PJqE97fQun_d4fAn5SmFBAeR3fO3SggHAQklQH8icQgMlExzOyByAilLUtZyR85yfR9UIpj6RGaVUgeLVnKzuhtRvMBXLfXDYWSx8TMXP0Ee7iZ1LwbTF7SH7obN9iF0RumKV4lPCnMMei9_DLplusC2aVKxMmw-fyUc_Tvxymhfk793yz81D-fjr_sfN9WNpBRd96WsngDbryjXUV2ptvWGOKyNk7RqUUjamUsKhqTivlK85M6oyIKxjjDIK_IJ8O97dpfgyYO71NmSLbWs6jEPWCiSTnNYjXByhTTHnhF7vUtiadNAU9NRQTw311FBPDceFy9PlYb1F985P0UZwdQImW9P6sYAN-c3VDVMwPVgfFY4V9gGTzjZMhV1IaHvtYvjfB_8AYpuNRA</recordid><startdate>20010301</startdate><enddate>20010301</enddate><creator>Albers, David S.</creator><creator>Swerdlow, Russell H.</creator><creator>Manfredi, Giovanni</creator><creator>Gajewski, Carl</creator><creator>Yang, Lichuan</creator><creator>Parker, W.Davis</creator><creator>Beal, M.Flint</creator><general>Elsevier Inc</general><general>Elsevier</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>7X8</scope></search><sort><creationdate>20010301</creationdate><title>Further Evidence for Mitochondrial Dysfunction in Progressive Supranuclear Palsy</title><author>Albers, David S. ; Swerdlow, Russell H. ; Manfredi, Giovanni ; Gajewski, Carl ; Yang, Lichuan ; Parker, W.Davis ; Beal, M.Flint</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c434t-f8d4019b5d91f57bcfa2d37a468d9e6669a574dea53357f832a75a04cd2212103</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2001</creationdate><topic>aconitase</topic><topic>Adenosine Triphosphate - metabolism</topic><topic>Aged</topic><topic>ATP</topic><topic>Biological and medical sciences</topic><topic>Blood Platelets - pathology</topic><topic>Blood Platelets - physiology</topic><topic>Cell Fusion</topic><topic>cybrids</topic><topic>Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases</topic><topic>Female</topic><topic>Humans</topic><topic>Hybrid Cells</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Mitochondria - metabolism</topic><topic>Neuroblastoma</topic><topic>neurodegeneration</topic><topic>Neurology</topic><topic>oxygen</topic><topic>Oxygen Consumption</topic><topic>Reference Values</topic><topic>Supranuclear Palsy, Progressive - blood</topic><topic>Supranuclear Palsy, Progressive - metabolism</topic><topic>Tumor Cells, Cultured</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Albers, David S.</creatorcontrib><creatorcontrib>Swerdlow, Russell H.</creatorcontrib><creatorcontrib>Manfredi, Giovanni</creatorcontrib><creatorcontrib>Gajewski, Carl</creatorcontrib><creatorcontrib>Yang, Lichuan</creatorcontrib><creatorcontrib>Parker, W.Davis</creatorcontrib><creatorcontrib>Beal, M.Flint</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>MEDLINE - Academic</collection><jtitle>Experimental neurology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Albers, David S.</au><au>Swerdlow, Russell H.</au><au>Manfredi, Giovanni</au><au>Gajewski, Carl</au><au>Yang, Lichuan</au><au>Parker, W.Davis</au><au>Beal, M.Flint</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Further Evidence for Mitochondrial Dysfunction in Progressive Supranuclear Palsy</atitle><jtitle>Experimental neurology</jtitle><addtitle>Exp Neurol</addtitle><date>2001-03-01</date><risdate>2001</risdate><volume>168</volume><issue>1</issue><spage>196</spage><epage>198</epage><pages>196-198</pages><issn>0014-4886</issn><eissn>1090-2430</eissn><coden>EXNEAC</coden><abstract>Recent data from our laboratory have identified a role for mitochondrial dysfunction in the pathogenesis of progressive supranuclear palsy (PSP). 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| subjects | aconitase Adenosine Triphosphate - metabolism Aged ATP Biological and medical sciences Blood Platelets - pathology Blood Platelets - physiology Cell Fusion cybrids Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases Female Humans Hybrid Cells Male Medical sciences Mitochondria - metabolism Neuroblastoma neurodegeneration Neurology oxygen Oxygen Consumption Reference Values Supranuclear Palsy, Progressive - blood Supranuclear Palsy, Progressive - metabolism Tumor Cells, Cultured |
| title | Further Evidence for Mitochondrial Dysfunction in Progressive Supranuclear Palsy |
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