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NF1 loss induces senescence during human melanocyte differentiation in an iPSC-based model

Neurofibromatosis type 1 (NF1) is a frequent genetic disease leading to the development of Schwann cell-derived neurofibromas or melanocytic lesions called café-au-lait macules (CALMs). The molecular mechanisms involved in CALMs formation remain largely unknown. In this report, we show for the firs...

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Hauptverfasser: Larribere, Lionel (VerfasserIn) , Wu, Huizi (VerfasserIn) , Novak, Daniel (VerfasserIn) , Galach, Marta (VerfasserIn) , Bernhardt, Mathias (VerfasserIn) , Orouji, Elias (VerfasserIn) , Weina, Kasia (VerfasserIn) , Knappe, Nathalie (VerfasserIn) , Umansky, Ludmila (VerfasserIn) , Beckhove, Philipp (VerfasserIn) , Umansky, Viktor (VerfasserIn) , Utikal, Jochen (VerfasserIn)
Dokumenttyp: Article (Journal)
Sprache:Englisch
Veröffentlicht: July 2015
In: Pigment cell & melanoma research
Year: 2015, Jahrgang: 28, Heft: 4, Pages: 407-416
ISSN:1755-148X
DOI:10.1111/pcmr.12369
Online-Zugang:Verlag, Volltext: http://dx.doi.org/10.1111/pcmr.12369
Verlag, Volltext: http://onlinelibrary.wiley.com/doi/10.1111/pcmr.12369/abstract
Volltext
Verfasserangaben:Lionel Larribere, Huizi Wu, Daniel Novak, Marta Galach, Mathias Bernhardt, Elias Orouji, Kasia Weina, Nathalie Knappe, Christos Sachpekidis, Ludmila Umansky, Philipp Beckhove, Viktor Umansky, Sofie De Schepper, Dieter Kaufmann, Robert Ballotti, Corine Bertolotto, Jochen Utikal
Beschreibung
Zusammenfassung:Neurofibromatosis type 1 (NF1) is a frequent genetic disease leading to the development of Schwann cell-derived neurofibromas or melanocytic lesions called café-au-lait macules (CALMs). The molecular mechanisms involved in CALMs formation remain largely unknown. In this report, we show for the first time pathophysiological mechanisms of abnormal melanocyte differentiation in a human NF1+/−-induced pluripotent stem cell (iPSC)-based model. We demonstrate that NF1 patient-derived fibroblasts can be successfully reprogrammed in NF1+/− iPSCs with active RAS signaling and that NF1 loss induces senescence during melanocyte differentiation as well as in patient's-derived CALMs, revealing a new role for NF1 in the melanocyte lineage.
Beschreibung:Gesehen am 07.04.2017
Beschreibung:Online Resource
ISSN:1755-148X
DOI:10.1111/pcmr.12369

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