A historical review of multiple system atrophy with a critical appraisal of cellular and animal models
Multiple system atrophy (MSA) is a progressive neurodegenerative disorder characterized by striatonigral degeneration (SND), olivopontocerebellar atrophy (OPCA), and dysautonomia with cerebellar ataxia or parkinsonian motor features. Isolated autonomic dysfunction with predominant genitourinary dysf...
Gespeichert in:
| Veröffentlicht in: | Journal of Neural Transmission 2021-10, Vol.128 (10), p.1507-1527 |
|---|---|
| Hauptverfasser: | , , |
| Format: | Artikel |
| Sprache: | eng |
| Schlagworte: | |
| Online-Zugang: | Volltext |
| Tags: |
Tag hinzufügen
Keine Tags, Fügen Sie den ersten Tag hinzu!
|
| container_end_page | 1527 |
|---|---|
| container_issue | 10 |
| container_start_page | 1507 |
| container_title | Journal of Neural Transmission |
| container_volume | 128 |
| creator | Marmion, David J. Peelaerts, Wouter Kordower, Jeffrey H. |
| description | Multiple system atrophy (MSA) is a progressive neurodegenerative disorder characterized by striatonigral degeneration (SND), olivopontocerebellar atrophy (OPCA), and dysautonomia with cerebellar ataxia or parkinsonian motor features. Isolated autonomic dysfunction with predominant genitourinary dysfunction and orthostatic hypotension and REM sleep behavior disorder are common characteristics of a prodromal phase, which may occur years prior to motor-symptom onset. MSA is a unique synucleinopathy, in which alpha-synuclein (aSyn) accumulates and forms insoluble inclusions in the cytoplasm of oligodendrocytes, termed glial cytoplasmic inclusions (GCIs). The origin of, and precise mechanism by which aSyn accumulates in MSA are unknown, and, therefore, disease-modifying therapies to halt or slow the progression of MSA are currently unavailable. For these reasons, much focus in the field is concerned with deciphering the complex neuropathological mechanisms by which MSA begins and progresses through the course of the disease. This review focuses on the history, etiopathogenesis, neuropathology, as well as cell and animal models of MSA. |
| doi_str_mv | 10.1007/s00702-021-02419-8 |
| format | Article |
| fullrecord | <record><control><sourceid>proquest_pubme</sourceid><recordid>TN_cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_8528759</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><sourcerecordid>2579633672</sourcerecordid><originalsourceid>FETCH-LOGICAL-c446t-1cc21f64dcad2d929fa10af3b935f19478dee83a30815dcc2bf67984be124de53</originalsourceid><addsrcrecordid>eNp9UctuHCEQRFaieG3nB3KIOOYyCa-ZgUsky3IekqVc4jNiofFiMcMEGFv798Ze20ouOTTdoquKpguhD5R8poSMX0o7COsIoy0EVZ08QhsqeN9RMfA3aEM4IZ3qB3GMTkq5JYRQOsp36JiLgXIhxQb5c7wLpaYcrIk4w12Ae5w8ntZYwxIBl32pMGFTc1p2e3wf6g4bbHOoTwyzLNmE0qpGshDjGk3GZnYtwtSup-QgljP01ptY4P1zPkXX3y5_X_zorn59_3lxftVZIYbaUWsZ9YNw1jjmFFPeUGI83yree6rEKB2A5IYTSXvXwFs_jEqKLVAmHPT8FH096C7rdgJnYa7ZRL3kNkve62SC_rczh52-SXda9kyOvWoCn54FcvqzQql6CuXxX2aGtBbN-lENnA8ja1B2gNqcSsngX5-hRD8apA8G6WaQfjJIy0b6-PeAr5QXRxqAHwClteYbyPo2rXluS_uf7AMoSZ6s</addsrcrecordid><sourcetype>Open Access Repository</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>2579633672</pqid></control><display><type>article</type><title>A historical review of multiple system atrophy with a critical appraisal of cellular and animal models</title><source>MEDLINE</source><source>SpringerLink Journals</source><creator>Marmion, David J. ; Peelaerts, Wouter ; Kordower, Jeffrey H.</creator><creatorcontrib>Marmion, David J. ; Peelaerts, Wouter ; Kordower, Jeffrey H.</creatorcontrib><description>Multiple system atrophy (MSA) is a progressive neurodegenerative disorder characterized by striatonigral degeneration (SND), olivopontocerebellar atrophy (OPCA), and dysautonomia with cerebellar ataxia or parkinsonian motor features. Isolated autonomic dysfunction with predominant genitourinary dysfunction and orthostatic hypotension and REM sleep behavior disorder are common characteristics of a prodromal phase, which may occur years prior to motor-symptom onset. MSA is a unique synucleinopathy, in which alpha-synuclein (aSyn) accumulates and forms insoluble inclusions in the cytoplasm of oligodendrocytes, termed glial cytoplasmic inclusions (GCIs). The origin of, and precise mechanism by which aSyn accumulates in MSA are unknown, and, therefore, disease-modifying therapies to halt or slow the progression of MSA are currently unavailable. For these reasons, much focus in the field is concerned with deciphering the complex neuropathological mechanisms by which MSA begins and progresses through the course of the disease. This review focuses on the history, etiopathogenesis, neuropathology, as well as cell and animal models of MSA.</description><identifier>ISSN: 0300-9564</identifier><identifier>EISSN: 1435-1463</identifier><identifier>DOI: 10.1007/s00702-021-02419-8</identifier><identifier>PMID: 34613484</identifier><language>eng</language><publisher>Vienna: Springer Vienna</publisher><subject>alpha-Synuclein ; Animals ; Inclusion Bodies ; Medicine ; Medicine & Public Health ; Models, Animal ; Multiple System Atrophy - pathology ; Nerve Degeneration - pathology ; Neurology ; Neurology and Preclinical Neurological Studies - Review ; Neurology and Preclinical Neurological Studies - Review Article ; Neurosciences ; Psychiatry</subject><ispartof>Journal of Neural Transmission, 2021-10, Vol.128 (10), p.1507-1527</ispartof><rights>The Author(s) 2021</rights><rights>2021. The Author(s).</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c446t-1cc21f64dcad2d929fa10af3b935f19478dee83a30815dcc2bf67984be124de53</citedby><cites>FETCH-LOGICAL-c446t-1cc21f64dcad2d929fa10af3b935f19478dee83a30815dcc2bf67984be124de53</cites><orcidid>0000-0001-8629-3294 ; 0000-0003-4575-2380 ; 0000-0003-3776-4162</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://link.springer.com/content/pdf/10.1007/s00702-021-02419-8$$EPDF$$P50$$Gspringer$$Hfree_for_read</linktopdf><linktohtml>$$Uhttps://link.springer.com/10.1007/s00702-021-02419-8$$EHTML$$P50$$Gspringer$$Hfree_for_read</linktohtml><link.rule.ids>230,314,776,780,881,27901,27902,41464,42533,51294</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/34613484$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Marmion, David J.</creatorcontrib><creatorcontrib>Peelaerts, Wouter</creatorcontrib><creatorcontrib>Kordower, Jeffrey H.</creatorcontrib><title>A historical review of multiple system atrophy with a critical appraisal of cellular and animal models</title><title>Journal of Neural Transmission</title><addtitle>J Neural Transm</addtitle><addtitle>J Neural Transm (Vienna)</addtitle><description>Multiple system atrophy (MSA) is a progressive neurodegenerative disorder characterized by striatonigral degeneration (SND), olivopontocerebellar atrophy (OPCA), and dysautonomia with cerebellar ataxia or parkinsonian motor features. Isolated autonomic dysfunction with predominant genitourinary dysfunction and orthostatic hypotension and REM sleep behavior disorder are common characteristics of a prodromal phase, which may occur years prior to motor-symptom onset. MSA is a unique synucleinopathy, in which alpha-synuclein (aSyn) accumulates and forms insoluble inclusions in the cytoplasm of oligodendrocytes, termed glial cytoplasmic inclusions (GCIs). The origin of, and precise mechanism by which aSyn accumulates in MSA are unknown, and, therefore, disease-modifying therapies to halt or slow the progression of MSA are currently unavailable. For these reasons, much focus in the field is concerned with deciphering the complex neuropathological mechanisms by which MSA begins and progresses through the course of the disease. This review focuses on the history, etiopathogenesis, neuropathology, as well as cell and animal models of MSA.</description><subject>alpha-Synuclein</subject><subject>Animals</subject><subject>Inclusion Bodies</subject><subject>Medicine</subject><subject>Medicine & Public Health</subject><subject>Models, Animal</subject><subject>Multiple System Atrophy - pathology</subject><subject>Nerve Degeneration - pathology</subject><subject>Neurology</subject><subject>Neurology and Preclinical Neurological Studies - Review</subject><subject>Neurology and Preclinical Neurological Studies - Review Article</subject><subject>Neurosciences</subject><subject>Psychiatry</subject><issn>0300-9564</issn><issn>1435-1463</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2021</creationdate><recordtype>article</recordtype><sourceid>C6C</sourceid><sourceid>EIF</sourceid><recordid>eNp9UctuHCEQRFaieG3nB3KIOOYyCa-ZgUsky3IekqVc4jNiofFiMcMEGFv798Ze20ouOTTdoquKpguhD5R8poSMX0o7COsIoy0EVZ08QhsqeN9RMfA3aEM4IZ3qB3GMTkq5JYRQOsp36JiLgXIhxQb5c7wLpaYcrIk4w12Ae5w8ntZYwxIBl32pMGFTc1p2e3wf6g4bbHOoTwyzLNmE0qpGshDjGk3GZnYtwtSup-QgljP01ptY4P1zPkXX3y5_X_zorn59_3lxftVZIYbaUWsZ9YNw1jjmFFPeUGI83yree6rEKB2A5IYTSXvXwFs_jEqKLVAmHPT8FH096C7rdgJnYa7ZRL3kNkve62SC_rczh52-SXda9kyOvWoCn54FcvqzQql6CuXxX2aGtBbN-lENnA8ja1B2gNqcSsngX5-hRD8apA8G6WaQfjJIy0b6-PeAr5QXRxqAHwClteYbyPo2rXluS_uf7AMoSZ6s</recordid><startdate>20211001</startdate><enddate>20211001</enddate><creator>Marmion, David J.</creator><creator>Peelaerts, Wouter</creator><creator>Kordower, Jeffrey H.</creator><general>Springer Vienna</general><scope>C6C</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><scope>5PM</scope><orcidid>https://orcid.org/0000-0001-8629-3294</orcidid><orcidid>https://orcid.org/0000-0003-4575-2380</orcidid><orcidid>https://orcid.org/0000-0003-3776-4162</orcidid></search><sort><creationdate>20211001</creationdate><title>A historical review of multiple system atrophy with a critical appraisal of cellular and animal models</title><author>Marmion, David J. ; Peelaerts, Wouter ; Kordower, Jeffrey H.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c446t-1cc21f64dcad2d929fa10af3b935f19478dee83a30815dcc2bf67984be124de53</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2021</creationdate><topic>alpha-Synuclein</topic><topic>Animals</topic><topic>Inclusion Bodies</topic><topic>Medicine</topic><topic>Medicine & Public Health</topic><topic>Models, Animal</topic><topic>Multiple System Atrophy - pathology</topic><topic>Nerve Degeneration - pathology</topic><topic>Neurology</topic><topic>Neurology and Preclinical Neurological Studies - Review</topic><topic>Neurology and Preclinical Neurological Studies - Review Article</topic><topic>Neurosciences</topic><topic>Psychiatry</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Marmion, David J.</creatorcontrib><creatorcontrib>Peelaerts, Wouter</creatorcontrib><creatorcontrib>Kordower, Jeffrey H.</creatorcontrib><collection>Springer Nature OA Free Journals</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><collection>PubMed Central (Full Participant titles)</collection><jtitle>Journal of Neural Transmission</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Marmion, David J.</au><au>Peelaerts, Wouter</au><au>Kordower, Jeffrey H.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>A historical review of multiple system atrophy with a critical appraisal of cellular and animal models</atitle><jtitle>Journal of Neural Transmission</jtitle><stitle>J Neural Transm</stitle><addtitle>J Neural Transm (Vienna)</addtitle><date>2021-10-01</date><risdate>2021</risdate><volume>128</volume><issue>10</issue><spage>1507</spage><epage>1527</epage><pages>1507-1527</pages><issn>0300-9564</issn><eissn>1435-1463</eissn><abstract>Multiple system atrophy (MSA) is a progressive neurodegenerative disorder characterized by striatonigral degeneration (SND), olivopontocerebellar atrophy (OPCA), and dysautonomia with cerebellar ataxia or parkinsonian motor features. Isolated autonomic dysfunction with predominant genitourinary dysfunction and orthostatic hypotension and REM sleep behavior disorder are common characteristics of a prodromal phase, which may occur years prior to motor-symptom onset. MSA is a unique synucleinopathy, in which alpha-synuclein (aSyn) accumulates and forms insoluble inclusions in the cytoplasm of oligodendrocytes, termed glial cytoplasmic inclusions (GCIs). The origin of, and precise mechanism by which aSyn accumulates in MSA are unknown, and, therefore, disease-modifying therapies to halt or slow the progression of MSA are currently unavailable. For these reasons, much focus in the field is concerned with deciphering the complex neuropathological mechanisms by which MSA begins and progresses through the course of the disease. This review focuses on the history, etiopathogenesis, neuropathology, as well as cell and animal models of MSA.</abstract><cop>Vienna</cop><pub>Springer Vienna</pub><pmid>34613484</pmid><doi>10.1007/s00702-021-02419-8</doi><tpages>21</tpages><orcidid>https://orcid.org/0000-0001-8629-3294</orcidid><orcidid>https://orcid.org/0000-0003-4575-2380</orcidid><orcidid>https://orcid.org/0000-0003-3776-4162</orcidid><oa>free_for_read</oa></addata></record> |
| fulltext | fulltext |
| identifier | ISSN: 0300-9564 |
| ispartof | Journal of Neural Transmission, 2021-10, Vol.128 (10), p.1507-1527 |
| issn | 0300-9564 1435-1463 |
| language | eng |
| recordid | cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_8528759 |
| source | MEDLINE; SpringerLink Journals |
| subjects | alpha-Synuclein Animals Inclusion Bodies Medicine Medicine & Public Health Models, Animal Multiple System Atrophy - pathology Nerve Degeneration - pathology Neurology Neurology and Preclinical Neurological Studies - Review Neurology and Preclinical Neurological Studies - Review Article Neurosciences Psychiatry |
| title | A historical review of multiple system atrophy with a critical appraisal of cellular and animal models |
| url | https://sfx.bib-bvb.de/sfx_tum?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2025-02-08T22%3A50%3A29IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-proquest_pubme&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=A%20historical%20review%20of%20multiple%20system%20atrophy%20with%20a%20critical%20appraisal%20of%20cellular%20and%20animal%20models&rft.jtitle=Journal%20of%20Neural%20Transmission&rft.au=Marmion,%20David%20J.&rft.date=2021-10-01&rft.volume=128&rft.issue=10&rft.spage=1507&rft.epage=1527&rft.pages=1507-1527&rft.issn=0300-9564&rft.eissn=1435-1463&rft_id=info:doi/10.1007/s00702-021-02419-8&rft_dat=%3Cproquest_pubme%3E2579633672%3C/proquest_pubme%3E%3Curl%3E%3C/url%3E&disable_directlink=true&sfx.directlink=off&sfx.report_link=0&rft_id=info:oai/&rft_pqid=2579633672&rft_id=info:pmid/34613484&rfr_iscdi=true |