Axial skeletal defects caused by mutation in the spondylocostal dysplasia/pudgy gene Dll3 are associated with disruption of the segmentation clock within the presomitic mesoderm
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Mutated MESP2 causes spondylocostal dysostosis in humans.Mutation of the LUNATIC FRINGE gene in humans causes spondylocostal dysostosis with a severe vertebral phenotypeTranscriptional oscillation of lunatic fringe is essential for somitogenesisNovel mutations in DLL3, a somitogenesis gene encoding a ligand for the Notch signalling pathway, cause a consistent pattern of abnormal vertebral segmentation in spondylocostal dysostosisProtein O-fucosyltransferase 1 is an essential component of Notch signaling pathwaysNotch regulation of bone development and remodeling and related skeletal disordersNotch signaling maintains neural rosette polarityNotchless is required for axial skeleton formation in micePhysiological notch signaling maintains bone homeostasis via RBPjk and Hey upstream of NFATc1Notch signaling in human development and diseaseO-fucosylation of DLL3 is required for its function during somitogenesisThe divergent DSL ligand Dll3 does not activate Notch signaling but cell autonomously attenuates signaling induced by other DSL ligandsPeriodic repression by the bHLH factor Hes7 is an essential mechanism for the somite segmentation clockThe T-box transcription factor Tbx18 maintains the separation of anterior and posterior somite compartmentsTie2Cre-mediated inactivation of plexinD1 results in congenital heart, vascular and skeletal defectsENU mutagenesis reveals that Notchless homolog 1 (Drosophila) affects Cdkn1a and several members of the Wnt pathway during murine pre-implantation developmentCharacterizing embryonic gene expression patterns in the mouse using nonredundant sequence-based selectionCompartmentalised expression of Delta-like 1 in epithelial somites is required for the formation of intervertebral joints.Comparison of pattern detection methods in microarray time series of the segmentation clock.Timing embryo segmentation: dynamics and regulatory mechanisms of the vertebrate segmentation clock.Expression of the oscillating gene her1 is directly regulated by Hairy/Enhancer of Split, T-box, and Suppressor of Hairless proteins in the zebrafish segmentation clock.Characterization of the skeletal fusion with sterility (sks) mouse showing axial skeleton abnormalities caused by defects of embryonic skeletal developmentTwo novel missense mutations in HAIRY-AND-ENHANCER-OF-SPLIT-7 in a family with spondylocostal dysostosis.A molecular and clinical study of Larsen syndrome caused by mutations in FLNBDevelopmental patterning of the cardiac atrioventricular canal by Notch and Hairy-related transcription factorsOsteogenic oxysterol, 20(S)-hydroxycholesterol, induces notch target gene expression in bone marrow stromal cells.The Her7 node modulates the network topology of the zebrafish segmentation clock via sequestration of the Hes6 hub.Notch signaling in skeletal health and disease.Comparison between Timelines of Transcriptional Regulation in Mammals, Birds, and Teleost Fish Somitogenesis.Divergent functions and distinct localization of the Notch ligands DLL1 and DLL3 in vivoMolecular genetics of congenital diaphragmatic defects.Anabolic actions of Notch on mature bone.Notch Signaling and the Skeleton.Physiological Notch signaling promotes gliogenesis in the developing peripheral and central nervous systemsCyclical expression of the Notch/Wnt regulator Nrarp requires modulation by Dll3 in somitogenesis.The many facets of Notch ligands.Genetic aspects of congenital and idiopathic scoliosis.Nas transgenic mouse line allows visualization of Notch pathway activity in vivo.Notch signaling maintains bone marrow mesenchymal progenitors by suppressing osteoblast differentiation.NOTCH signaling in Sertoli cells regulates gonocyte fate
P2860
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P2860
Axial skeletal defects caused by mutation in the spondylocostal dysplasia/pudgy gene Dll3 are associated with disruption of the segmentation clock within the presomitic mesoderm
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2002 nî lūn-bûn
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2002 թուականի Ապրիլին հրատարակուած գիտական յօդուած
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2002 թվականի ապրիլին հրատարակված գիտական հոդված
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2002年の論文
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2002年論文
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2002年論文
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2002年論文
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2002年論文
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2002年論文
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2002年论文
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name
Axial skeletal defects caused ...... within the presomitic mesoderm
@ast
Axial skeletal defects caused ...... within the presomitic mesoderm
@en
Axial skeletal defects caused ...... within the presomitic mesoderm
@nl
type
label
Axial skeletal defects caused ...... within the presomitic mesoderm
@ast
Axial skeletal defects caused ...... within the presomitic mesoderm
@en
Axial skeletal defects caused ...... within the presomitic mesoderm
@nl
prefLabel
Axial skeletal defects caused ...... within the presomitic mesoderm
@ast
Axial skeletal defects caused ...... within the presomitic mesoderm
@en
Axial skeletal defects caused ...... within the presomitic mesoderm
@nl
P2093
P3181
P1433
P1476
Axial skeletal defects caused ...... within the presomitic mesoderm
@en
P2093
Duncan B Sparrow
Melanie Clements
Ronald A Conlon
Rosa S P Beddington
Sally L Dunwoodie
P304
P3181
P407
P577
2002-04-01T00:00:00Z