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  Partial blindness to submicron topography in NF1 haploinsufficient cultured fibroblasts indicates a new function of neurofibromin in regulation of mechanosensoric

Kaufmann, D., Hoesch, J., Su, Y., Deeg, L., Mellert, K., Spatz, J. P., et al. (2012). Partial blindness to submicron topography in NF1 haploinsufficient cultured fibroblasts indicates a new function of neurofibromin in regulation of mechanosensoric. Molecular Syndromology, 3(4), 169-179. doi:10.1159/000342698.

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Item Permalink: http://hdl.handle.net/11858/00-001M-0000-0010-4BC4-8 Version Permalink: http://hdl.handle.net/11858/00-001M-0000-002E-5DE8-5
Genre: Journal Article

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 Creators:
Kaufmann, Dieter, Author              
Hoesch, J., Author
Su, Y., Author              
Deeg, L., Author
Mellert, K., Author
Spatz, Joachim P.1, 2, Author              
Kemkemer, Ralf1, Author              
Affiliations:
1Cellular Biophysics, Max Planck Institute for Medical Research, Max Planck Society, ou_2364731              
2Biophysical Chemistry, Institute of Physical Chemistry, University of Heidelberg, 69120 Heidelberg, Germany, ou_persistent22              

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Free keywords: Mechanosensoric; Neurofibromatosis type 1; Neurofibromin; Topography
 Abstract: Cells sense physical properties of their extracellular environment and translate them into biochemical signals. In this study, cell responses to surfaces with submicron topographies were investigated in cultured human NF1 haploinsufficient fibroblasts. Age-matched fibroblasts from 8 patients with neurofibromatosis type 1 (NF1(+/-)) and 9 controls (NF1(+/+)) were cultured on surfaces with grooves of 200 nm height and lateral distance of 2 μm. As cellular response indicator, the mean cell orientation along microstructured grooves was systematically examined. The tested NF1 haploinsufficient fibroblasts were significantly less affected by the topography than those from healthy donors. Incubation of the NF1(+/-) fibroblasts with the farnesyltransferase inhibitor FTI-277 and other inhibitors of the neurofibromin pathway ameliorates significantly the cell orientation. These data indicate that NF1 haploinsufficiency results in an altered response to specific surface topography in fibroblasts. We suggest a new function of neurofibromin in the sensoric mechanism to topographies and a partial mechanosensoric blindness by NF1 haploinsufficiency.

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Language(s): deu - German
 Dates: 2012-08-082012-09-272012-10-01
 Publication Status: Published in print
 Pages: 11
 Publishing info: -
 Table of Contents: -
 Rev. Type: Peer
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Title: Molecular Syndromology
Source Genre: Journal
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Publ. Info: Basel : S. Karger
Pages: - Volume / Issue: 3 (4) Sequence Number: - Start / End Page: 169 - 179 Identifier: ISSN: 1661-8769