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Skin-Derived Precursor Cells as an In Vitro Modelling Tool for the Study of Type 1 Neurofibromatosis
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 ev...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Hindawi Publishing Corporation
2012
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3329859/ https://www.ncbi.nlm.nih.gov/pubmed/22550514 http://dx.doi.org/10.1155/2012/646725 |
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author | Gutiérrez-Rivera, Araika Iribar, Haizea Tuneu, Anna Izeta, Ander |
author_facet | Gutiérrez-Rivera, Araika Iribar, Haizea Tuneu, Anna Izeta, Ander |
author_sort | Gutiérrez-Rivera, Araika |
collection | PubMed |
description | 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 S100β 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. |
format | Online Article Text |
id | pubmed-3329859 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2012 |
publisher | Hindawi Publishing Corporation |
record_format | MEDLINE/PubMed |
spelling | pubmed-33298592012-05-01 Skin-Derived Precursor Cells as an In Vitro Modelling Tool for the Study of Type 1 Neurofibromatosis Gutiérrez-Rivera, Araika Iribar, Haizea Tuneu, Anna Izeta, Ander Stem Cells Int Review Article 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 S100β 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. Hindawi Publishing Corporation 2012 2012-04-01 /pmc/articles/PMC3329859/ /pubmed/22550514 http://dx.doi.org/10.1155/2012/646725 Text en Copyright © 2012 Araika Gutiérrez-Rivera et al. https://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Review Article Gutiérrez-Rivera, Araika Iribar, Haizea Tuneu, Anna Izeta, Ander Skin-Derived Precursor Cells as an In Vitro Modelling Tool for the Study of Type 1 Neurofibromatosis |
title | Skin-Derived Precursor Cells as an In Vitro Modelling Tool for the Study of Type 1 Neurofibromatosis |
title_full | Skin-Derived Precursor Cells as an In Vitro Modelling Tool for the Study of Type 1 Neurofibromatosis |
title_fullStr | Skin-Derived Precursor Cells as an In Vitro Modelling Tool for the Study of Type 1 Neurofibromatosis |
title_full_unstemmed | Skin-Derived Precursor Cells as an In Vitro Modelling Tool for the Study of Type 1 Neurofibromatosis |
title_short | Skin-Derived Precursor Cells as an In Vitro Modelling Tool for the Study of Type 1 Neurofibromatosis |
title_sort | skin-derived precursor cells as an in vitro modelling tool for the study of type 1 neurofibromatosis |
topic | Review Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3329859/ https://www.ncbi.nlm.nih.gov/pubmed/22550514 http://dx.doi.org/10.1155/2012/646725 |
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