Multiple system atrophy: A primary oligodendrogliopathy
Identifieur interne : 001046 ( Main/Corpus ); précédent : 001045; suivant : 001047Multiple system atrophy: A primary oligodendrogliopathy
Auteurs : Gregor K. Wenning ; Nadia Stefanova ; Kurt A. Jellinger ; Werner Poewe ; Michael G. SchlossmacherSource :
- Annals of Neurology [ 0364-5134 ] ; 2008-09-28.
Abstract
To this day, the cause of multiple system atrophy (MSA) remains stubbornly enigmatic. A growing body of observations regarding the clinical, morphological, and biochemical phenotypes of MSA has been published, but the interested student is still left without a clue as to its underlying cause. MSA has long been considered a rare cousin of Parkinson's disease and cerebellar degeneration; it is rich in acronyms but poor in genetic and environmental leads. Because of the worldwide research efforts conducted over the last two decades and the discovery of the α‐synuclein–encoding SNCA gene as a cause of rare familial Parkinson's disease, the MSA field has seen advances on three fronts: the identification of its principal cellular target, that is, oligodendrocytes; the characterization of α‐synuclein–rich glial cytoplasmic inclusions as a suitable marker at autopsy; and improved diagnostic accuracy in living patients resulting from detailed clinicopathological studies. The working model of MSA as a primary glial disorder was recently strengthened by the finding of dysregulation in the metabolism of myelin basic protein and p25α, a central nervous system–specific phosphoprotein (also called tubulin polymerization promoting protein, TPPP). Intriguingly, in early cases of MSA, the oligodendrocytic changes in myelin basic protein and p25α processing were recorded even before formation of glial cytoplasmic inclusions became detectable. Here, we review the evolving concept that MSA may not just be related to Parkinson's disease but also share traits with the family of demyelinating disorders. Although these syndromes vary in their respective cause of oligodendrogliopathy, they have in common myelin disruption that is often followed by axonal dysfunction. Ann Neurol 2008;64:239–246
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DOI: 10.1002/ana.21465
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A growing body of observations regarding the clinical, morphological, and biochemical phenotypes of MSA has been published, but the interested student is still left without a clue as to its underlying cause. MSA has long been considered a rare cousin of Parkinson's disease and cerebellar degeneration; it is rich in acronyms but poor in genetic and environmental leads. Because of the worldwide research efforts conducted over the last two decades and the discovery of the α‐synuclein–encoding SNCA gene as a cause of rare familial Parkinson's disease, the MSA field has seen advances on three fronts: the identification of its principal cellular target, that is, oligodendrocytes; the characterization of α‐synuclein–rich glial cytoplasmic inclusions as a suitable marker at autopsy; and improved diagnostic accuracy in living patients resulting from detailed clinicopathological studies. The working model of MSA as a primary glial disorder was recently strengthened by the finding of dysregulation in the metabolism of myelin basic protein and p25α, a central nervous system–specific phosphoprotein (also called tubulin polymerization promoting protein, TPPP). Intriguingly, in early cases of MSA, the oligodendrocytic changes in myelin basic protein and p25α processing were recorded even before formation of glial cytoplasmic inclusions became detectable. Here, we review the evolving concept that MSA may not just be related to Parkinson's disease but also share traits with the family of demyelinating disorders. Although these syndromes vary in their respective cause of oligodendrogliopathy, they have in common myelin disruption that is often followed by axonal dysfunction. Ann Neurol 2008;64:239–246</div></front></TEI><istex><corpusName>wiley</corpusName><author><json:item><name>Gregor K. 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Because of the worldwide research efforts conducted over the last two decades and the discovery of the α‐synuclein–encoding SNCA gene as a cause of rare familial Parkinson's disease, the MSA field has seen advances on three fronts: the identification of its principal cellular target, that is, oligodendrocytes; the characterization of α‐synuclein–rich glial cytoplasmic inclusions as a suitable marker at autopsy; and improved diagnostic accuracy in living patients resulting from detailed clinicopathological studies. The working model of MSA as a primary glial disorder was recently strengthened by the finding of dysregulation in the metabolism of myelin basic protein and p25α, a central nervous system–specific phosphoprotein (also called tubulin polymerization promoting protein, TPPP). Intriguingly, in early cases of MSA, the oligodendrocytic changes in myelin basic protein and p25α processing were recorded even before formation of glial cytoplasmic inclusions became detectable. Here, we review the evolving concept that MSA may not just be related to Parkinson's disease but also share traits with the family of demyelinating disorders. Although these syndromes vary in their respective cause of oligodendrogliopathy, they have in common myelin disruption that is often followed by axonal dysfunction. 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The working model of MSA as a primary glial disorder was recently strengthened by the finding of dysregulation in the metabolism of myelin basic protein and p25α, a central nervous system–specific phosphoprotein (also called tubulin polymerization promoting protein, TPPP). Intriguingly, in early cases of MSA, the oligodendrocytic changes in myelin basic protein and p25α processing were recorded even before formation of glial cytoplasmic inclusions became detectable. Here, we review the evolving concept that MSA may not just be related to Parkinson's disease but also share traits with the family of demyelinating disorders. Although these syndromes vary in their respective cause of oligodendrogliopathy, they have in common myelin disruption that is often followed by axonal dysfunction. 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USA</unparsedAffiliation></affiliation></affiliationGroup><fundingInfo><fundingAgency>Austrian Science Foundation</fundingAgency><fundingNumber>DK‐C34‐B05</fundingNumber><fundingNumber>P19989‐B05</fundingNumber></fundingInfo><fundingInfo><fundingAgency>MSA Fund at Brigham and Women's Hospital</fundingAgency><fundingNumber>GM‐2004</fundingNumber></fundingInfo><fundingInfo><fundingAgency>NIH (National Institute of Neurological Disorders and Stroke)</fundingAgency><fundingNumber>P50 NS38375</fundingNumber></fundingInfo><abstractGroup><abstract type="main" xml:lang="en"><title type="main">Abstract</title><p>To this day, the cause of multiple system atrophy (MSA) remains stubbornly enigmatic. A growing body of observations regarding the clinical, morphological, and biochemical phenotypes of MSA has been published, but the interested student is still left without a clue as to its underlying cause. MSA has long been considered a rare cousin of Parkinson's disease and cerebellar degeneration; it is rich in acronyms but poor in genetic and environmental leads. Because of the worldwide research efforts conducted over the last two decades and the discovery of the α‐synuclein–encoding <i>SNCA</i> gene as a cause of rare familial Parkinson's disease, the MSA field has seen advances on three fronts: the identification of its principal cellular target, that is, oligodendrocytes; the characterization of α‐synuclein–rich glial cytoplasmic inclusions as a suitable marker at autopsy; and improved diagnostic accuracy in living patients resulting from detailed clinicopathological studies. The working model of MSA as a primary glial disorder was recently strengthened by the finding of dysregulation in the metabolism of myelin basic protein and p25α, a central nervous system–specific phosphoprotein (also called tubulin polymerization promoting protein, TPPP). Intriguingly, in early cases of MSA, the oligodendrocytic changes in myelin basic protein and p25α processing were recorded even before formation of glial cytoplasmic inclusions became detectable. Here, we review the evolving concept that MSA may not just be related to Parkinson's disease but also share traits with the family of demyelinating disorders. Although these syndromes vary in their respective cause of oligodendrogliopathy, they have in common myelin disruption that is often followed by axonal dysfunction. Ann Neurol 2008;64:239–246</p></abstract></abstractGroup></contentMeta></header></component></istex:document></istex:metadataXml><mods version="3.6"><titleInfo lang="en"><title>Multiple system atrophy: A primary oligodendrogliopathy</title></titleInfo><titleInfo type="abbreviated" lang="en"><title>An Oligodendrogliopathy</title></titleInfo><titleInfo type="alternative" contentType="CDATA" lang="en"><title>Multiple system atrophy: A primary oligodendrogliopathy</title></titleInfo><name type="personal"><namePart type="given">Gregor K.</namePart><namePart type="family">Wenning</namePart><namePart type="termsOfAddress">MD, MSc, PhD</namePart><affiliation>Department of Neurology, Innsbruck Medical University, Innsbruck</affiliation><description>Correspondence: Parkinson and Movement Disorder Centre, Department of Neurology, Medical University Innsbruck, Anichstrasse 35, 6020 Innsbruck, Austria</description><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Nadia</namePart><namePart type="family">Stefanova</namePart><namePart type="termsOfAddress">MD, PhD</namePart><affiliation>Department of Neurology, Innsbruck Medical University, Innsbruck</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Kurt A.</namePart><namePart type="family">Jellinger</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Institute of Clinical Neurobiology, Vienna, Austria</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Werner</namePart><namePart type="family">Poewe</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Neurology, Innsbruck Medical University, Innsbruck</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Michael G.</namePart><namePart type="family">Schlossmacher</namePart><namePart type="termsOfAddress">MD, FRCPC</namePart><affiliation>Division of Neuroscience, Ottawa Health Research Institute, University of Ottawa, Ottawa, Ontario, Canada</affiliation><affiliation>Department of Neurology, Brigham & Women's Hospital and Harvard Medical School, Boston, Massachusetts, USA</affiliation><role><roleTerm type="text">author</roleTerm></role></name><typeOfResource>text</typeOfResource><genre type="review-article" displayLabel="reviewArticle"></genre><originInfo><publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher><place><placeTerm type="text">Hoboken</placeTerm></place><dateIssued encoding="w3cdtf">2008-09-28</dateIssued><dateCaptured encoding="w3cdtf">2008-01-27</dateCaptured><dateValid encoding="w3cdtf">2008-06-13</dateValid><copyrightDate encoding="w3cdtf">2008</copyrightDate></originInfo><language><languageTerm type="code" authority="rfc3066">en</languageTerm><languageTerm type="code" authority="iso639-2b">eng</languageTerm></language><physicalDescription><internetMediaType>text/html</internetMediaType><extent unit="figures">1</extent><extent unit="tables">1</extent><extent unit="references">93</extent><extent unit="words">5808</extent></physicalDescription><abstract lang="en">To this day, the cause of multiple system atrophy (MSA) remains stubbornly enigmatic. A growing body of observations regarding the clinical, morphological, and biochemical phenotypes of MSA has been published, but the interested student is still left without a clue as to its underlying cause. MSA has long been considered a rare cousin of Parkinson's disease and cerebellar degeneration; it is rich in acronyms but poor in genetic and environmental leads. Because of the worldwide research efforts conducted over the last two decades and the discovery of the α‐synuclein–encoding SNCA gene as a cause of rare familial Parkinson's disease, the MSA field has seen advances on three fronts: the identification of its principal cellular target, that is, oligodendrocytes; the characterization of α‐synuclein–rich glial cytoplasmic inclusions as a suitable marker at autopsy; and improved diagnostic accuracy in living patients resulting from detailed clinicopathological studies. The working model of MSA as a primary glial disorder was recently strengthened by the finding of dysregulation in the metabolism of myelin basic protein and p25α, a central nervous system–specific phosphoprotein (also called tubulin polymerization promoting protein, TPPP). Intriguingly, in early cases of MSA, the oligodendrocytic changes in myelin basic protein and p25α processing were recorded even before formation of glial cytoplasmic inclusions became detectable. Here, we review the evolving concept that MSA may not just be related to Parkinson's disease but also share traits with the family of demyelinating disorders. Although these syndromes vary in their respective cause of oligodendrogliopathy, they have in common myelin disruption that is often followed by axonal dysfunction. Ann Neurol 2008;64:239–246</abstract><note type="funding">Austrian Science Foundation - No. DK‐C34‐B05; No. P19989‐B05; </note><note type="funding">MSA Fund at Brigham and Women's Hospital - No. GM‐2004; </note><note type="funding">NIH (National Institute of Neurological Disorders and Stroke) - No. P50 NS38375; </note><relatedItem type="host"><titleInfo><title>Annals of Neurology</title><subTitle>Official Journal of the American Neurological Association and the Child Neurology Society</subTitle></titleInfo><titleInfo type="abbreviated"><title>Ann Neurol.</title></titleInfo><genre type="Journal">journal</genre><subject><genre>article category</genre><topic>Point of View</topic></subject><identifier type="ISSN">0364-5134</identifier><identifier type="eISSN">1531-8249</identifier><identifier type="DOI">10.1002/(ISSN)1531-8249</identifier><identifier type="PublisherID">ANA</identifier><part><date>2008</date><detail type="volume"><caption>vol.</caption><number>64</number></detail><detail type="issue"><caption>no.</caption><number>3</number></detail><extent unit="pages"><start>239</start><end>246</end><total>8</total></extent></part></relatedItem><identifier type="istex">8D91885D6CCE8CC9DCE26077C20D7EE1974D04AF</identifier><identifier type="DOI">10.1002/ana.21465</identifier><identifier type="ArticleID">ANA21465</identifier><accessCondition type="use and reproduction" contentType="copyright">Copyright © 2008 American Neurological Association</accessCondition><recordInfo><recordContentSource>WILEY</recordContentSource><recordOrigin>Wiley Subscription Services, Inc., A Wiley Company</recordOrigin></recordInfo></mods></metadata><serie></serie></istex></record>Pour manipuler ce document sous Unix (Dilib)
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