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Conference Paper: Sox10 mutation affects enteric neural crest cell migration in a cell autonomous manner

TitleSox10 mutation affects enteric neural crest cell migration in a cell autonomous manner
Authors
KeywordsBiology
Issue Date2010
PublisherElsevier Ltd. The Journal's web site is located at http://www.elsevier.com/wps/find/journaldescription.cws_home/717204/description#description
Citation
The 16th International Conference on the International Society of Differentiation, Nara, Japan, 15–18 November 2010. In Differentiation, 2010, v. 80 suppl. 1, p. S23, abstract P18 How to Cite?
AbstractEnteric neural crest stem cells (eNCSCs) are a pluripotent, migratory cell population that gives rise to the enteric neurons and glia throughout the gastrointestinal tract. Sox10 is a transcription factor required for the maintenance of pluripotency and survival of eNCSCs during enteric nervous system (ENS) development. SOX10 mutations disrupt NCSCs development and result in Waardenburg–Hirschsprung (WS4) disease, which displays varying degrees of intestinal aganglionosis, pigmentation and hearing defects. To elucidate the role of Sox10 in migration of eNCSCs, and to understand how the impaired function contributes to gut aganglionosis, we generated a novel WS4 mouse mutant Sox10NGFP where EGFP reporter was fused to the N-terminal region of Sox10. EGFP expression in Sox10NGFP mice recaptured the dynamics of Sox10 expression during development. Neurospheres of mutant eNCSCs exhibited reduced proliferation capability as demonstrated by BrdU incorporation assay. The mean size of mutant neurospheres was significantly reduced compared with wildtype control. Using time-lapse imaging, we found that migratory eNCSCs in Sox10NGFP/+ mutants displayed altered trajectories and reduced migration speed compared to Wnt1cre/ZEG control. Explant culture, assays that determine the advancement of eENCCs in normal/abnormal gut environment, indicated that migration defects in Sox10NGFP/+ mice occurred in a cell-autonomous manner. Together, these findings suggested that Sox10 mutation impaired the proliferation and migration capability of eNCSCs. The abnormal migration behavior of mutant eNCSCs restricted the colonization of eNCSC along the gut wall, and contributed to aganglionosis in mutant guts.
DescriptionThis journal suppl. contain abstracts of the The 16th International Conference on the International Society of Differentiation ... 2010.
Persistent Identifierhttp://hdl.handle.net/10722/133382
ISSN
2015 Impact Factor: 2.461
2015 SCImago Journal Rankings: 1.747
ISI Accession Number ID

 

DC FieldValueLanguage
dc.contributor.authorZhang, Men_US
dc.contributor.authorLeung, Cen_US
dc.contributor.authorLui, VCHen_US
dc.contributor.authorTam, PKHen_US
dc.contributor.authorSham, MHen_US
dc.date.accessioned2011-05-11T08:33:59Z-
dc.date.available2011-05-11T08:33:59Z-
dc.date.issued2010en_US
dc.identifier.citationThe 16th International Conference on the International Society of Differentiation, Nara, Japan, 15–18 November 2010. In Differentiation, 2010, v. 80 suppl. 1, p. S23, abstract P18en_US
dc.identifier.issn0301-4681-
dc.identifier.urihttp://hdl.handle.net/10722/133382-
dc.descriptionThis journal suppl. contain abstracts of the The 16th International Conference on the International Society of Differentiation ... 2010.-
dc.description.abstractEnteric neural crest stem cells (eNCSCs) are a pluripotent, migratory cell population that gives rise to the enteric neurons and glia throughout the gastrointestinal tract. Sox10 is a transcription factor required for the maintenance of pluripotency and survival of eNCSCs during enteric nervous system (ENS) development. SOX10 mutations disrupt NCSCs development and result in Waardenburg–Hirschsprung (WS4) disease, which displays varying degrees of intestinal aganglionosis, pigmentation and hearing defects. To elucidate the role of Sox10 in migration of eNCSCs, and to understand how the impaired function contributes to gut aganglionosis, we generated a novel WS4 mouse mutant Sox10NGFP where EGFP reporter was fused to the N-terminal region of Sox10. EGFP expression in Sox10NGFP mice recaptured the dynamics of Sox10 expression during development. Neurospheres of mutant eNCSCs exhibited reduced proliferation capability as demonstrated by BrdU incorporation assay. The mean size of mutant neurospheres was significantly reduced compared with wildtype control. Using time-lapse imaging, we found that migratory eNCSCs in Sox10NGFP/+ mutants displayed altered trajectories and reduced migration speed compared to Wnt1cre/ZEG control. Explant culture, assays that determine the advancement of eENCCs in normal/abnormal gut environment, indicated that migration defects in Sox10NGFP/+ mice occurred in a cell-autonomous manner. Together, these findings suggested that Sox10 mutation impaired the proliferation and migration capability of eNCSCs. The abnormal migration behavior of mutant eNCSCs restricted the colonization of eNCSC along the gut wall, and contributed to aganglionosis in mutant guts.-
dc.languageengen_US
dc.publisherElsevier Ltd. The Journal's web site is located at http://www.elsevier.com/wps/find/journaldescription.cws_home/717204/description#description-
dc.relation.ispartofDifferentiationen_US
dc.rightsNOTICE: this is the author’s version of a work that was accepted for publication in <Journal title>. Changes resulting from the publishing process, such as peer review, editing, corrections, structural formatting, and other quality control mechanisms may not be reflected in this document. Changes may have been made to this work since it was submitted for publication. A definitive version was subsequently published in PUBLICATION, [VOL#, ISSUE#, (DATE)] DOI#-
dc.subjectBiology-
dc.titleSox10 mutation affects enteric neural crest cell migration in a cell autonomous manneren_US
dc.typeConference_Paperen_US
dc.identifier.emailLui, VCH: vchlui@hku.hken_US
dc.identifier.emailTam, PKH: paultam@hku.hken_US
dc.identifier.emailSham, MH: mhsham@hku.hken_US
dc.identifier.authorityLui, VCH=rp00363en_US
dc.identifier.authorityTam, PKH=rp00060en_US
dc.identifier.authoritySham, MH=rp00380en_US
dc.identifier.doi10.1016/j.diff.2010.09.024-
dc.identifier.hkuros184998en_US
dc.identifier.volume80en_US
dc.identifier.issuesuppl. 1-
dc.identifier.spageS23en_US
dc.identifier.epageS23en_US
dc.identifier.isiWOS:000283589700056-
dc.publisher.placeUnited Kingdom-

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