Research Topics
Genomes and Genes | Veronique PingaultSummaryCountry: France Publications
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Publications
Review and update of mutations causing Waardenburg syndromeVeronique Pingault
Inserm Unite U955, Département de génétique, Laboratoire de Biochimie et Génétique, Hopital Henri Mondor, Creteil, France
Hum Mutat 31:391-406. 2010..In this review we provide an update on all WS genes and set up mutation databases, summarize molecular and functional data available for each of them, and discuss the applications in diagnostics and genetic counseling...
Screening of MITF and SOX10 regulatory regions in Waardenburg syndrome type 2Viviane Baral
INSERM, U955, equipe11, Creteil, France
PLoS ONE 7:e41927. 2012..Functional analyses argued against a pathogenic effect of these variations, suggesting that mutations within regulatory elements of WS genes are not a major cause of this neurocristopathy...
Deletions at the SOX10 gene locus cause Waardenburg syndrome types 2 and 4Nadege Bondurand
INSERM U841, Institut Mondor de Recherche Biomedicale, Département de génétique, Universite Paris 12, Paris, France
Am J Hum Genet 81:1169-85. 2007..This study further characterizes the molecular complexity and the close relationship that links the different subtypes of WS...
Interactions between Sox10, Edn3 and Ednrb during enteric nervous system and melanocyte developmentLaure Stanchina
INSERM, U654, Bases moléculaires et cellulaires des maladies génétiques, Hopital Henri Mondor, Creteil, F 94000, France Université Paris 12, Faculte de Medecine, IFR10, Creteil, F 94000, France
Dev Biol 295:232-49. 2006..These data will contribute to the understanding of the molecular basis of ENS, pigmentation and hearing defects observed in mouse mutants and patients carrying SOX10, EDN3 and EDNRB mutations...
Alu-mediated deletion of SOX10 regulatory elements in Waardenburg syndrome type 4Nadege Bondurand
INSERM, U955, equipe11, Hopital Henri Mondor, Creteil, France
Eur J Hum Genet 20:990-4. 2012..This opens up new routes to the molecular dissection of neural crest disorders...
Identification and functional analysis of SOX10 missense mutations in different subtypes of Waardenburg syndromeAsma Chaoui
INSERM U955, Hopital Henri Mondor, Creteil, France
Hum Mutat 32:1436-49. 2011..Whether this phenomenon is a cause or a consequence of mutation-associated pathogenicity remains to be determined, but this observation could help to identify new SOX10 modes of action...
SOX10 mutations in chronic intestinal pseudo-obstruction suggest a complex physiopathological mechanismVeronique Pingault
INSERM U468, Génétique Moléculaire et Physiopathologie, and Laboratoire de Biochimie, Hopital Henri Mondor, 94010 Creteil Cedex, France
Hum Genet 111:198-206. 2002..These results show that chronic intestinal pseudo-obstruction may be a manifestation associated with WS, and indicate that aganglionosis is not the only mechanism underlying the intestinal dysfunction of patients with SOX10 mutations...
