53 results match your criteria: "Centre de Reference des Mastocytoses[Affiliation]"
Pediatric mastocytosis is a clonal disease associated with D816V and other activating c-KIT mutations.
J Invest Dermatol
March 2010
Service de Dermatologie, Hôpital Necker, CEREMAST, Centre de Référence des Mastocytoses, Faculté Necker, Université René Descartes Paris V, Paris, France.
Adult mastocytosis is an incurable clonal disease associated with c-KIT mutations, mostly in exon 17 (D816V). In contrast, pediatric mastocytosis often spontaneously regresses and is considered a reactive disease. Previous studies on childhood mastocytosis assessed only a few patients and focused primarily on codon 816 mutations, with various results.
View Article and Find Full Text PDFMasitinib (AB1010), a potent and selective tyrosine kinase inhibitor targeting KIT.
PLoS One
September 2009
INSERM, U891, Centre de Recherche en Cancérologie de Marseille, Signalisation, Hematopoiesis and Mechanisms of Oncogenesis, Centre de référence des mastocytoses, Marseille, France.
Background: The stem cell factor receptor, KIT, is a target for the treatment of cancer, mastocytosis, and inflammatory diseases. Here, we characterise the in vitro and in vivo profiles of masitinib (AB1010), a novel phenylaminothiazole-type tyrosine kinase inhibitor that targets KIT.
Methodology/principal Findings: In vitro, masitinib had greater activity and selectivity against KIT than imatinib, inhibiting recombinant human wild-type KIT with an half inhibitory concentration (IC(50)) of 200+/-40 nM and blocking stem cell factor-induced proliferation and KIT tyrosine phosphorylation with an IC(50) of 150+/-80 nM in Ba/F3 cells expressing human or mouse wild-type KIT.
Phenotypic and genotypic characteristics of mastocytosis according to the age of onset.
PLoS One
April 2008
Université Paris V, Service de maladies infectieuses et tropicales, Centre de référence des mastocytoses, Hôpital Necker Enfants malades, Centre d'Infectiologie Necker-Pasteur, Paris, France.
Article Synopsis
- Adult mastocytosis tends to have ongoing systemic involvement with a higher prevalence of c-kit 816 mutations, while pediatric mastocytosis is usually skin-limited and can resolve on its own.
- A study of 142 adult patients revealed that systemic disease percentages were similar in those with childhood-onset compared to adult-onset mastocytosis, but a significant difference in c-kit mutation rates was observed.
- The findings suggest that the development of mastocytosis varies based on age at onset, which could be crucial for future c-kit-targeted treatments.