{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T01:42:19Z","timestamp":1791250939909,"version":"4.1.0"},"reference-count":37,"publisher":"Society for Neuroscience","issue":"35","license":[{"start":{"date-parts":[[2012,2,29]],"date-time":"2012-02-29T00:00:00Z","timestamp":1330473600000},"content-version":"vor","delay-in-days":182,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-sa\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J. Neurosci."],"published-print":{"date-parts":[[2011,8,31]]},"abstract":"<jats:p>\n                    Mutations in the methyl-CpG-binding protein 2 (MeCP2) result in Rett syndrome (RTT), an X-linked disorder that disrupts neurodevelopment. Girls with RTT exhibit motor deficits similar to those in Parkinson's disease, suggesting defects in the nigrostriatal pathway. This study examined age-dependent changes in dopamine neurons of the substantia nigra (SN) from wild-type, presymptomatic, and symptomatic\n                    <jats:italic>Mecp2<\/jats:italic>\n                    <jats:sup>+\/\u2212<\/jats:sup>\n                    mice. Mecp2\n                    <jats:sup>+<\/jats:sup>\n                    neurons in the SN in\n                    <jats:italic>Mecp2<\/jats:italic>\n                    <jats:sup>+\/\u2212<\/jats:sup>\n                    mice were indistinguishable in morphology, resting conductance, and dopamine current density from neurons in wild-type mice. However, the capacitance, total dendritic length, and resting conductance of Mecp2\n                    <jats:sup>\u2212<\/jats:sup>\n                    neurons were less than those of Mecp2\n                    <jats:sup>+<\/jats:sup>\n                    neurons as early as 4 weeks after birth, before overt symptoms. These differences were maintained throughout life. In symptomatic\n                    <jats:italic>Mecp2<\/jats:italic>\n                    <jats:sup>+\/\u2212<\/jats:sup>\n                    mice, the current induced by activation of D\n                    <jats:sub>2<\/jats:sub>\n                    dopamine autoreceptors was significantly less in Mecp2\n                    <jats:sup>\u2212<\/jats:sup>\n                    neurons than in Mecp2\n                    <jats:sup>+<\/jats:sup>\n                    neurons, although D\n                    <jats:sub>2<\/jats:sub>\n                    receptor density was unaltered in\n                    <jats:italic>Mecp2<\/jats:italic>\n                    <jats:sup>+\/\u2212<\/jats:sup>\n                    mice. Electrochemical measurements revealed that significantly less dopamine was released after stimulation of striatum in adult\n                    <jats:italic>Mecp2<\/jats:italic>\n                    <jats:sup>+\/\u2212<\/jats:sup>\n                    mice compared to wild type. The decrease in size and function of Mecp2\n                    <jats:sup>\u2212<\/jats:sup>\n                    neurons observed in adult\n                    <jats:italic>Mecp2<\/jats:italic>\n                    <jats:sup>+\/\u2212<\/jats:sup>\n                    mice was recapitulated in dopamine neurons from symptomatic\n                    <jats:italic>Mecp2<\/jats:italic>\n                    <jats:sup>\u2212\/y<\/jats:sup>\n                    males. These results show that mutation in\n                    <jats:italic>Mecp2<\/jats:italic>\n                    results in cell-autonomous defects in the SN early in life and throughout adulthood. Ultimately, dysfunction in terminal dopamine release and D\n                    <jats:sub>2<\/jats:sub>\n                    autoreceptor-dependent currents in dopamine neurons from symptomatic females support the idea that decreased dopamine transmission due to heterogeneous Mecp2 expression contributes to the parkinsonian features of RTT in\n                    <jats:italic>Mecp2<\/jats:italic>\n                    <jats:sup>+\/\u2212<\/jats:sup>\n                    mice.\n                  <\/jats:p>","DOI":"10.1523\/jneurosci.0684-11.2011","type":"journal-article","created":{"date-parts":[[2011,8,31]],"date-time":"2011-08-31T20:30:55Z","timestamp":1314822655000},"page":"12629-12637","source":"Crossref","is-referenced-by-count":58,"title":["Loss of Mecp2 in Substantia Nigra Dopamine Neurons Compromises the Nigrostriatal Pathway"],"prefix":"10.1523","volume":"31","author":[{"given":"Stephanie 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