Cellular Origin, Tumor Progression, and Pathogenic Mechanisms of Cutaneous Neurofibromas Revealed by Mice with Nf1 Knockout in Boundary Cap Cells

边界帽细胞中 Nf1 基因敲除的小鼠揭示皮肤神经纤维瘤的细胞起源、肿瘤进展和致病机制

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作者:Katarzyna J Radomska, Fanny Coulpier, Aurelie Gresset, Alain Schmitt, Amal Debbiche, Sophie Lemoine, Pierre Wolkenstein, Jean-Michel Vallat, Patrick Charnay, Piotr Topilko

Significance

Patients affected by NF1 develop numerous cNFs. We present a mouse model that faithfully recapitulates cNFs, identify a candidate cell type at their origin, analyze the steps involved in their formation, and show that their development is dramatically accelerated by skin injury. These findings have important clinical/therapeutic implications.This article is highlighted in the In This Issue feature, p. 1.

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