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Karger AG","issue":"4","license":[{"start":{"date-parts":[[2012,1,1]],"date-time":"2012-01-01T00:00:00Z","timestamp":1325376000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"},{"start":{"date-parts":[[2012,1,1]],"date-time":"2012-01-01T00:00:00Z","timestamp":1325376000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Dev Neurosci"],"published-print":{"date-parts":[[2012]]},"abstract":"<jats:p>Early-onset generalized torsion dystonia, also known as DYT1 dystonia, is a childhood onset heritable neurological movement disorder involving painful, involuntary muscle contractions, sustained abnormal postures, and repetitive movements. It is caused by a GAG deletion in the &lt;i&gt;Tor1A&lt;\/i&gt; gene located on chromosome 9. TorsinA, the product of the &lt;i&gt;Tor1A&lt;\/i&gt; gene, is expressed throughout the brain beginning early in embryonic development. It plays a role in the regulation of nuclear envelope-cytoskeletal interactions, and presumably nuclear translocation. Since nuclear translocation, powered by cytoskeletal traction, is critical for cell proliferation and migration, we examined whether neurogenesis and neuronal migration are affected in &lt;i&gt;Tor1A\u2013&lt;\/i&gt;\/&lt;i&gt;\u2013&lt;\/i&gt; mouse brain. Our data show that interkinetic nuclear migration and the pattern of migration of newly generated neurons are impaired in the dorsal forebrain of the &lt;i&gt;Tor1A\u2013&lt;\/i&gt;\/&lt;i&gt;\u2013&lt;\/i&gt; embryo. However, neurogenesis is not altered significantly. The rate of migration of cells from explants of the medial ganglionic eminence is also impaired in the &lt;i&gt;Tor1A\u2013&lt;\/i&gt;\/&lt;i&gt;\u2013&lt;\/i&gt; embryo. Thus, loss of torsinA results in subtle but significant alterations in cell proliferation and migration in the embryonic forebrain. These subtle developmental changes are consistent with a lack of significant changes in neuronal numbers, neuronal positioning or size of brain regions in DYT1 dystonia patients.<\/jats:p>","DOI":"10.1159\/000342260","type":"journal-article","created":{"date-parts":[[2012,9,27]],"date-time":"2012-09-27T01:53:49Z","timestamp":1348710829000},"page":"366-378","source":"Crossref","is-referenced-by-count":19,"title":["Neurogenesis and Neuronal Migration in the Forebrain of the TorsinA Knockout Mouse Embryo"],"prefix":"10.1159","volume":"34","author":[{"given":"Deirdre 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