{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,10]],"date-time":"2026-10-10T16:23:32Z","timestamp":1791649412104,"version":"4.3.4"},"reference-count":29,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2017,9,26]],"date-time":"2017-09-26T00:00:00Z","timestamp":1506384000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2017,9,26]],"date-time":"2017-09-26T00:00:00Z","timestamp":1506384000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/100000065","name":"National Institute of Neurological Disorders and Stroke","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000065","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Movement Disorders"],"published-print":{"date-parts":[[2017,12]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    <jats:bold>Background<\/jats:bold>\n                    : Recent neuroimaging studies implicate nigrostriatal degeneration as a critical factor in producing late\u2010onset parkinsonism in patients with\n                    <jats:sc>l<\/jats:sc>\n                    \u2010dopa\u2010responsive dystonia\u2010causing mutations. However, postmortem anatomical studies do not reveal neurodegeneration in\n                    <jats:sc>l<\/jats:sc>\n                    \u2010dopa\u2010responsive dystonia patients. These contrasting findings make it unclear how parkinsonism develops in\n                    <jats:sc>l<\/jats:sc>\n                    \u2010dopa\u2010responsive dystonia mutation carriers.\n                  <\/jats:p>\n                  <jats:p>\n                    <jats:bold>Methods<\/jats:bold>\n                    : We prospectively assessed motor dysfunction, responses to dopaminergic challenge, and dopamine neuron degeneration with aging in a validated knockin mouse model bearing a\n                    <jats:sc>l<\/jats:sc>\n                    \u2010dopa\u2010responsive dystonia\u2010causing mutation found in humans.\n                  <\/jats:p>\n                  <jats:p>\n                    <jats:bold>Results<\/jats:bold>\n                    : As\n                    <jats:sc>l<\/jats:sc>\n                    \u2010dopa\u2010responsive dystonia mice aged, dystonic movements waned while locomotor activity decreased and initiation of movements slowed. Despite the age\u2010related reduction in movement, there was no evidence for degeneration of midbrain dopamine neurons. Presynaptically mediated dopaminergic responses did not change with age in\n                    <jats:sc>l<\/jats:sc>\n                    \u2010dopa\u2010responsive dystonia mice, but responses to D1 dopamine receptor agonists decreased with age.\n                  <\/jats:p>\n                  <jats:p>\n                    <jats:bold>Conclusions<\/jats:bold>\n                    : We have demonstrated for the first time the co\u2010occurrence of dystonia and Parkinson's\u2010like features (mainly consisting of hypokinesia) in a genetic mouse model. In this model we show that these features evolve without dopaminergic neurodegeneration, suggesting that postsynaptic plasticity, rather than presynaptic degeneration, may contribute to the development of parkinsonism in patients with\n                    <jats:sc>l<\/jats:sc>\n                    \u2010dopa\u2010responsive dystonia. \u00a9 2017 International Parkinson and Movement Disorder Society\n                  <\/jats:p>","DOI":"10.1002\/mds.27169","type":"journal-article","created":{"date-parts":[[2017,9,26]],"date-time":"2017-09-26T07:35:10Z","timestamp":1506411310000},"page":"1694-1700","source":"Crossref","is-referenced-by-count":17,"title":["Parkinsonism without dopamine neuron degeneration in aged\n                    <scp>l<\/scp>\n                    \u2010dopa\u2010responsive dystonia knockin mice"],"prefix":"10.1002","volume":"32","author":[{"given":"Samuel J.","family":"Rose","sequence":"first","affiliation":[{"name":"Department of Pharmacology Emory University School of Medicine Atlanta Georgia USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Porter","family":"Harrast","sequence":"additional","affiliation":[{"name":"Department of Pharmacology Emory University School of Medicine Atlanta Georgia USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christine","family":"Donsante","sequence":"additional","affiliation":[{"name":"Department of Pharmacology Emory University School of Medicine Atlanta Georgia USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xueliang","family":"Fan","sequence":"additional","affiliation":[{"name":"Department of Pharmacology Emory University School of Medicine Atlanta Georgia USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valerie","family":"Joers","sequence":"additional","affiliation":[{"name":"Department of Physiology Emory University School of Medicine Atlanta Georgia USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mal\u00fa G.","family":"Tansey","sequence":"additional","affiliation":[{"name":"Department of Physiology Emory University School of Medicine Atlanta Georgia USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H.A.","family":"Jinnah","sequence":"additional","affiliation":[{"name":"Department of Neurology Emory University School of Medicine Atlanta Georgia USA"},{"name":"Department of Human Genetics Emory University School of Medicine Atlanta Georgia USA"},{"name":"Department of Pediatrics Emory University School of Medicine Atlanta Georgia USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ellen J.","family":"Hess","sequence":"additional","affiliation":[{"name":"Department of Pharmacology Emory University School of Medicine Atlanta Georgia USA"},{"name":"Department of Neurology Emory University School of Medicine Atlanta Georgia USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2017,9,26]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(10)70269-6"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1038\/nrneurol.2015.86"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410350405"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.53.5.1032"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awu179"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neurobiolaging.2016.10.006"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.parkreldis.2015.01.004"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/4.7.1209"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awv212"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.21829"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/B978-0-12-405195-9.00004-4"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbd.2012.05.009"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0169-328X(00)00194-7"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1402134111"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.3233\/JPD-140410"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneurol.2012.574"},{"key":"e_1_2_6_18_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.2008.155861"},{"key":"e_1_2_6_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1353-8020(00)00084-5"},{"key":"e_1_2_6_20_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.41.2_Part_1.174"},{"key":"e_1_2_6_21_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.942800"},{"key":"e_1_2_6_22_1","doi-asserted-by":"publisher","DOI":"10.1002\/humu.22565"},{"key":"e_1_2_6_23_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0099592"},{"key":"e_1_2_6_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0197-4580(02)00232-4"},{"key":"e_1_2_6_25_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0019415"},{"key":"e_1_2_6_26_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0033-1337337"},{"key":"e_1_2_6_27_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000003810"},{"key":"e_1_2_6_28_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuroimage.2015.07.035"},{"key":"e_1_2_6_29_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0022754"},{"key":"e_1_2_6_30_1","doi-asserted-by":"publisher","DOI":"10.1124\/pr.115.012260"}],"container-title":["Movement 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