| The development of cutaneous neurofibromas. | |
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MedLine Citation:
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PMID: 21281783 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Cutaneous neurofibromas are the hallmarks of neurofibromatosis type 1 (NF1). They are composed of multiple cell types, and traditionally they are believed to arise from small nerve tributaries of the skin. A key finding in the context of this view has been that subpopulations of tumor Schwann cells harbor biallelic inactivation of the NF1 gene (NF1(-/-)). In the present study, our aim was to clarify further the pathogenesis of cutaneous neurofibromas. First, we detected cells expressing multipotency-associated biomarkers in cutaneous neurofibromas. Second, we developed a method for isolating and expanding multipotent neurofibroma-derived precursor cells (NFPs) from dissociated human cutaneous neurofibromas and used it to analyze their growth and differentiation potential. In analogy to solitary cells resident in neurofibromas, NFPs were found to express nestin and had the potential to differentiate to, at least, Schwann cells, neurons, epithelial cells, and adipocytes. Mutation analysis of the NFPs revealed that their genotype was NF1(+/-). The results led us to speculate that the development of cutaneous neurofibromas includes the recruitment of multipotent NF1(+/-) precursor cells. These cells may be derived from the multipotent cells of the hair roots, which often are intimately associated with microscopic neurofibromas. |
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Authors:
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Eeva-Mari Jouhilahti; Sirkku Peltonen; Tom Callens; Elina Jokinen; Anthony M Heape; Ludwine Messiaen; Juha Peltonen |
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Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: The American journal of pathology Volume: 178 ISSN: 1525-2191 ISO Abbreviation: Am. J. Pathol. Publication Date: 2011 Feb |
Date Detail:
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Created Date: 2011-02-01 Completed Date: 2011-05-23 Revised Date: 2013-04-12 |
Medline Journal Info:
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Nlm Unique ID: 0370502 Medline TA: Am J Pathol Country: United States |
Other Details:
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Languages: eng Pagination: 500-5 Citation Subset: AIM; IM |
Copyright Information:
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Copyright © 2011 American Society for Investigative Pathology. Published by Elsevier Inc. All rights reserved. |
Affiliation:
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Department of Cell Biology and Anatomy, Institute of Biomedicine, University of Turku, Turku, Finland. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Cell Differentiation DNA Mutational Analysis Hair Follicle / pathology Humans Multipotent Stem Cells / metabolism Neurofibroma / genetics, pathology* Neurofibromin 1 / genetics Precancerous Conditions / pathology* Skin Neoplasms / genetics, pathology* Tumor Cells, Cultured Tumor Markers, Biological / metabolism |
| Chemical | |
Reg. No./Substance:
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0/Neurofibromin 1; 0/Tumor Markers, Biological |
| Comments/Corrections | |
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