Mesenchymal stem cell dysfunction in HGPS

A model for mesenchymal stem cell dysfunction in HGPS. In wild-type mesenchymal stem cells (MSC; left), Notch signaling, which operates through the cleavable and nuclear-penetrating Notch intracellular domain (NICD), is active at basal levels, resulting in basal expression of Notch downstream targets, including Hes1, Hes3, Hes5, Hey1, Hey2, and TLE1. In Hutchison-Gilford progeria syndrome (HGPS), Notch signaling is increased in mesenchymal stem cells (right), leading to an increase in the expression of Notch pathway genes and to perturbed differentiation, including increased osteogenesis and decreased adipogenesis. INM, inner nuclear membrane.
Mesenchymal stem cell dysfunction in HGPS
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Publication

Gone with the Wnt/Notch: stem cells in laminopathies, progeria, and aging. (2008) Eran Meshorer, et al. J Cell Biol. 2008 Apr 7;181(1):9-13. Figure: F2.

Gene mentions


Organism Group Word Match Source NCBI Symbol NCBI ID
Homo sapiens Primates Notch NOTCH famplex_relations NOTCH1 4851
Homo sapiens Primates Notch NOTCH famplex_relations NOTCH2 4853
Homo sapiens Primates Notch NOTCH famplex_relations NOTCH3 4854
Homo sapiens Primates Notch NOTCH famplex_relations NOTCH4 4855
Homo sapiens Primates Hes1 HES1 ncbigene_symbol HES1 3280
Homo sapiens Primates Hes1 HES1 ncbigene_synonym GATD3 8209
Homo sapiens Primates Hey1/2 HEY2 ncbigene_symbol HEY2 23493
Homo sapiens Primates Hey1/2 HEY1 ncbigene_symbol HEY1 23462
Homo sapiens Primates Hes1/3/5 HES5 ncbigene_symbol HES5 388585
Homo sapiens Primates Hes1/3/5 HES3 ncbigene_symbol HES3 390992
Homo sapiens Primates TLE1 TLE1 ncbigene_symbol TLE1 7088
Homo sapiens Primates bok BOK ncbigene_symbol BOK 666
Homo sapiens Primates MSC MSC ncbigene_symbol MSC 9242
Homo sapiens Primates MSC MSC ncbigene_synonym SLC25A37 51312
Homo sapiens Primates HGPS HGPS ncbigene_synonym LMNA 4000
Homo sapiens Primates HGPS HGPS ncbigene_synonym ZMPSTE24 10269
Homo sapiens Primates HGPS HGPS ncbigene_synonym ROBO3 64221

Chemical mentions

Word Match MeSH Name ChEBI

Disease mentions

Word Match MeSH Name DOID