Skin-Derived Precursor Cells as an In Vitro Modelling Tool for the Study of Type 1 Neurofibromatosis. (1st April 2012)

• Record Type: Journal Article
• Authors: Gutiérrez-Rivera, Araika; Iribar, Haizea; Tuneu, Anna; Izeta, Ander
• Other Names: Familari Mary Academic Editor.
• Is Part Of: Stem cells international. Volume 2012(2012)
• Publication Date: 2012-04-01
• Subjects: Stem Cells -- Periodicals; Stem Cells -- Therapeutic use -- Periodicals; Stem Cells -- Transplantation -- Periodicals; 616.0277405
• DOI: 10.1155/2012/646725
• Languages: English
• ISSNs: 1687-966X

Abstract

Abstract : The most characteristic feature of neurofibromatosis type 1 (NF1) is the development of neurofibromas. It has been suggested that these tumors are caused by somatic inactivation of the wild-type NF1 allele, but the cell that originally suffers this mutation remains controversial. Several lines of evidence support the clonal origin of these tumors, and it has been recently suggested that skin-derived precursor cells (SKPs) could be the cell of origin of dermal neurofibromas. Nullizygous ( NF1 −/− ) SKPs do give rise to neurofibromas when transplanted to heterozygous mice. Moreover, a nullizygous population of cells that is S100 β negative is present in human neurofibromas, and NF1 +/− multipotent progenitor cells are seemingly recruited to the tumor. This evidence supports the neurofibroma stem cell hypothesis and a putative involvement of SKPs in the aetiopathogenesis of the disease, suggesting that SKPs could become a valuable tool for the in vitro study of NF1.