{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T07:46:56Z","timestamp":1792741616341,"version":"4.5.0"},"reference-count":43,"publisher":"Cold Spring Harbor Laboratory","issue":"1","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Genes Dev."],"published-print":{"date-parts":[[2001,1,1]]},"abstract":"<jats:p>\n                    The molecular mechanisms that determine glial cell fate in the vertebrate nervous system have not been elucidated. Peripheral glial cells differentiate from pluripotent neural crest cells. We show here that the transcription factor Sox10 is a key regulator in differentiation of peripheral glial cells. In mice that carry a spontaneous or a targeted mutation of\n                    <jats:italic>Sox10<\/jats:italic>\n                    , neuronal cells form in dorsal root ganglia, but Schwann cells or satellite cells are not generated. At later developmental stages, this lack of peripheral glial cells results in a severe degeneration of sensory and motor neurons. Moreover, we show that Sox10 controls expression of\n                    <jats:italic>ErbB3<\/jats:italic>\n                    in neural crest cells.\n                    <jats:italic>ErbB3<\/jats:italic>\n                    encodes a Neuregulin receptor, and down-regulation of\n                    <jats:italic>ErbB3<\/jats:italic>\n                    accounts for many changes in development of neural crest cells observed in\n                    <jats:italic>Sox10<\/jats:italic>\n                    mutant mice. Sox10 also has functions not mediated by ErbB3, for instance in the melanocyte lineage. Phenotypes observed in heterozygous mice that carry a targeted\n                    <jats:italic>Sox10<\/jats:italic>\n                    null allele reproduce those observed in heterozygous\n                    <jats:italic>Sox10<\/jats:italic>\n                    <jats:sup>\n                      <jats:italic>Dom<\/jats:italic>\n                    <\/jats:sup>\n                    mice. Haploinsufficiency of\n                    <jats:italic>Sox10<\/jats:italic>\n                    can thus cause pigmentation and megacolon defects, which are also observed in\n                    <jats:italic>Sox10<\/jats:italic>\n                    <jats:sup>\n                      <jats:italic>Dom<\/jats:italic>\n                    <\/jats:sup>\n                    \/+ mice and in patients with Waardenburg-Hirschsprung disease caused by heterozygous\n                    <jats:italic>SOX10<\/jats:italic>\n                    mutations.\n                  <\/jats:p>","DOI":"10.1101\/gad.186601","type":"journal-article","created":{"date-parts":[[2002,7,26]],"date-time":"2002-07-26T20:00:34Z","timestamp":1027713634000},"page":"66-78","source":"Crossref","is-referenced-by-count":846,"title":["The transcription factor Sox10 is a key regulator of peripheral glial development"],"prefix":"10.1101","volume":"15","author":[{"given":"Stefan","family":"Britsch","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Derk E.","family":"Goerich","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dieter","family":"Riethmacher","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Reto I.","family":"Peirano","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Moritz","family":"Rossner","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Klaus-Armin","family":"Nave","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carmen","family":"Birchmeier","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Wegner","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"246","published-online":{"date-parts":[[2001,1,1]]},"reference":[{"key":"2021111507315395000_15.1.66.1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1098-1136(20000115)29:2<104::AID-GLIA2>3.0.CO;2-2"},{"key":"2021111507315395000_15.1.66.2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/8.9.1785"},{"key":"2021111507315395000_15.1.66.3","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/9.13.1907"},{"key":"2021111507315395000_15.1.66.4","doi-asserted-by":"publisher","DOI":"10.1101\/gad.12.12.1825"},{"key":"2021111507315395000_15.1.66.5","doi-asserted-by":"publisher","DOI":"10.1006\/geno.1995.1212"},{"key":"2021111507315395000_15.1.66.6","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(91)90071-6"},{"key":"2021111507315395000_15.1.66.7","doi-asserted-by":"crossref","first-page":"4999","DOI":"10.1242\/dev.124.24.4999","article-title":"ErbB3 is required for normal cerebellar and cardiac development: A comparison with ErbB2-and heregulin-deficient mice.","volume":"124","author":"Erickson","year":"1997","journal-title":"Development"},{"key":"2021111507315395000_15.1.66.8","doi-asserted-by":"publisher","DOI":"10.1016\/0165-3806(93)90163-5"},{"key":"2021111507315395000_15.1.66.9","doi-asserted-by":"publisher","DOI":"10.1002\/1521-1878(200011)22:11<987::AID-BIES5>3.0.CO;2-5"},{"key":"2021111507315395000_15.1.66.10","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(88)90020-7"},{"key":"2021111507315395000_15.1.66.11","doi-asserted-by":"publisher","DOI":"10.1101\/gad.14.14.1712"},{"key":"2021111507315395000_15.1.66.12","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.95.9.5161"},{"key":"2021111507315395000_15.1.66.13","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(90)90303-V"},{"key":"2021111507315395000_15.1.66.14","doi-asserted-by":"publisher","DOI":"10.1002\/1531-8249(199909)46:3<313::AID-ANA6>3.0.CO;2-7"},{"key":"2021111507315395000_15.1.66.15","doi-asserted-by":"crossref","first-page":"527","DOI":"10.1002\/j.1460-2075.1992.tb05083.x","article-title":"A second tyrosinase-related protein, TRP-2, maps to and is mutated at the mouse slaty locus.","volume":"11","author":"Jackson","year":"1992","journal-title":"EMBO J."},{"key":"2021111507315395000_15.1.66.16","doi-asserted-by":"publisher","DOI":"10.1126\/science.273.5274.507"},{"key":"2021111507315395000_15.1.66.17","doi-asserted-by":"publisher","DOI":"10.1016\/S0959-437X(97)80014-7"},{"key":"2021111507315395000_15.1.66.18","doi-asserted-by":"publisher","DOI":"10.1007\/s100249900162"},{"key":"2021111507315395000_15.1.66.19","doi-asserted-by":"crossref","first-page":"237","DOI":"10.1523\/JNEUROSCI.18-01-00237.1998","article-title":"Sox10, a novel transcriptional modulator in glial cells.","volume":"18","author":"Kuhlbrodt","year":"1998","journal-title":"J. Neurosci."},{"key":"2021111507315395000_15.1.66.20","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.273.36.23033"},{"key":"2021111507315395000_15.1.66.21","doi-asserted-by":"crossref","first-page":"2637","DOI":"10.1242\/dev.120.9.2637","article-title":"The expression pattern of a novel gene encoding brain-fatty acid binding protein correlates with neuronal and glial cell development.","volume":"120","author":"Kurtz","year":"1994","journal-title":"Development"},{"key":"2021111507315395000_15.1.66.22","doi-asserted-by":"crossref","first-page":"435","DOI":"10.1093\/oxfordjournals.jhered.a109980","article-title":"Association of megacolon with a new dominant spotting gene (Dom) in the mouse.","volume":"75","author":"Lane","year":"1984","journal-title":"J. Hered."},{"key":"2021111507315395000_15.1.66.23","doi-asserted-by":"crossref","unstructured":"Le Douarin N. Kalcheim C. (1999) The neural crest. (Cambridge University Press, Cambridge, UK).","DOI":"10.1017\/CBO9780511897948"},{"key":"2021111507315395000_15.1.66.24","doi-asserted-by":"publisher","DOI":"10.1038\/378386a0"},{"key":"2021111507315395000_15.1.66.25","doi-asserted-by":"crossref","first-page":"3575","DOI":"10.1242\/dev.124.18.3575","article-title":"Isoform-specific expression and function of neuregulin.","volume":"124","author":"Meyer","year":"1997","journal-title":"Development"},{"key":"2021111507315395000_15.1.66.26","doi-asserted-by":"publisher","DOI":"10.1016\/S0896-6273(00)80779-5"},{"key":"2021111507315395000_15.1.66.27","doi-asserted-by":"publisher","DOI":"10.1016\/S0092-8674(00)80860-0"},{"key":"2021111507315395000_15.1.66.28","doi-asserted-by":"publisher","DOI":"10.1093\/nar\/28.16.3047"},{"key":"2021111507315395000_15.1.66.29","doi-asserted-by":"publisher","DOI":"10.1128\/MCB.20.9.3198-3209.2000"},{"key":"2021111507315395000_15.1.66.30","doi-asserted-by":"publisher","DOI":"10.1038\/ng0298-171"},{"key":"2021111507315395000_15.1.66.31","doi-asserted-by":"publisher","DOI":"10.1002\/1531-8249(200010)48:4<671::AID-ANA17>3.0.CO;2-8"},{"key":"2021111507315395000_15.1.66.32","doi-asserted-by":"publisher","DOI":"10.1007\/s004390050793"},{"key":"2021111507315395000_15.1.66.33","doi-asserted-by":"publisher","DOI":"10.1038\/39593"},{"key":"2021111507315395000_15.1.66.34","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(94)90150-3"},{"key":"2021111507315395000_15.1.66.35","doi-asserted-by":"publisher","DOI":"10.1038\/ng0198-60"},{"key":"2021111507315395000_15.1.66.36","doi-asserted-by":"crossref","first-page":"215","DOI":"10.1101\/gr.9.3.215","article-title":"The Sox10(Dom) mouse: Modeling the genetic variation of Waardenburg- Shah (WS4) syndrome.","volume":"9","author":"Southard-Smith","year":"1999","journal-title":"Genome Res."},{"key":"2021111507315395000_15.1.66.37","doi-asserted-by":"publisher","DOI":"10.1038\/371796a0"},{"key":"2021111507315395000_15.1.66.38","doi-asserted-by":"publisher","DOI":"10.1086\/302895"},{"key":"2021111507315395000_15.1.66.39","doi-asserted-by":"crossref","first-page":"2811","DOI":"10.1242\/dev.127.13.2811","article-title":"Fate determination of neural crest cells by NOTCH-mediated lateral inhibition and asymmetrical cell division during gangliogenesis.","volume":"127","author":"Wakamatsu","year":"2000","journal-title":"Development"},{"key":"2021111507315395000_15.1.66.40","doi-asserted-by":"publisher","DOI":"10.1093\/nar\/27.6.1409"},{"key":"2021111507315395000_15.1.66.41","doi-asserted-by":"crossref","first-page":"199","DOI":"10.1242\/dev.113.1.199","article-title":"A homolog of Drosophila Notch expressed during mammalian development.","volume":"113","author":"Weinmaster","year":"1991","journal-title":"Development"},{"key":"2021111507315395000_15.1.66.42","doi-asserted-by":"publisher","DOI":"10.1101\/gad.13.19.2538"},{"key":"2021111507315395000_15.1.66.43","doi-asserted-by":"publisher","DOI":"10.1016\/S0896-6273(00)80873-9"}],"container-title":["Genes &amp; Development"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1101\/gad.186601","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,4,23]],"date-time":"2023-04-23T02:45:25Z","timestamp":1682217925000},"score":1,"resource":{"primary":{"URL":"http:\/\/genesdev.cshlp.org\/lookup\/doi\/10.1101\/gad.186601"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2001,1,1]]},"references-count":43,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2001,1,1]]}},"alternative-id":["10.1101\/gad.186601"],"URL":"https:\/\/doi.org\/10.1101\/gad.186601","relation":{},"ISSN":["0890-9369","1549-5477"],"issn-type":[{"value":"0890-9369","type":"print"},{"value":"1549-5477","type":"electronic"}],"subject":[],"published":{"date-parts":[[2001,1,1]]}}}