English
 
User Manual Privacy Policy Disclaimer Contact us
  Advanced SearchBrowse

Item

ITEM ACTIONSEXPORT
  Deterioration of fracture healing in the mouse model of NF1 long bone dysplasia

El Khassawna, T., Toben, D., Kolanczyk, M., Schmidt-Bleek, K., Koennecke, I., Schell, H., et al. (2012). Deterioration of fracture healing in the mouse model of NF1 long bone dysplasia. Bone, 51(4), 651-660. doi:10.1016/j.bone.2012.07.011.

Item is

Basic

show hide
Item Permalink: http://hdl.handle.net/11858/00-001M-0000-000E-F264-A Version Permalink: http://hdl.handle.net/11858/00-001M-0000-000E-F265-8
Genre: Journal Article

Files

show Files
hide Files
:
Khassawna.pdf (Publisher version), 4MB
Name:
Khassawna.pdf
Description:
-
Visibility:
Public
MIME-Type / Checksum:
application/pdf / [MD5]
Technical Metadata:
Copyright Date:
-
Copyright Info:
© 2012 Elsevier Inc.
License:
-

Locators

show

Creators

show
hide
 Creators:
El Khassawna, T., Author
Toben, D., Author
Kolanczyk, M.1, 2, Author              
Schmidt-Bleek, K., Author
Koennecke, I., Author
Schell, H., Author
Mundlos, S.1, 2, 3, Author              
Duda, G. N., Author
Affiliations:
1Research Group Development & Disease (Head: Stefan Mundlos), Max Planck Institute for Molecular Genetics, Max Planck Society, Berlin, Germany, ou_1433557              
2Institute for Medical Genetics, Charité-Universitätsmedizin Berlin, Berlin, Germany, ou_persistent22              
3Berlin Brandenburg Center for Regenerative Therapies (BCRT), Berlin, Germany, ou_persistent22              

Content

show
hide
Free keywords: Actins/metabolism Animals Bone Diseases, Developmental/genetics/ pathology Disease Models, Animal Fracture Healing Genes, Neurofibromatosis 1 Male Mice Mice, Inbred C57BL Mice, Knockout Osteoblasts/pathology Tibia/ pathology/radiography Tomography, X-Ray Computed
 Abstract: Neurofibromatosis type 1 (NF1) is an autosomal dominant genetic disease resulting from inactivating mutations in the gene encoding the protein neurofibromin. NF1 manifests as a heritable susceptibility to tumours of neural tissue mainly located in the skin (neurofibromas) and pigmented skin lesions. Besides these more common clinical manifestations, many NF1 patients (50%) have abnormalities of the skeleton. Long bones are often affected (usually the tibia) and the clinical signs range from bowing to spontaneous fractures and non-unions. Here we present the analysis of bone fracture healing in the Nf1(Prx1)-knock-out mouse, a model of NF1 long bone dysplasia. In line with previously reported cortical bone injury results, fracture healing was impaired in Nf1(Prx1) mice. We showed that the defective fracture healing in Nf1(Prx1) mice is characterized by diminished cartilaginous callus formation and a thickening of the periosteal bone. These changes are paralleled by fibrous tissue accumulation within the fracture site. We identify a population of fibrous tissue cells within the Nf1 deficient fracture as alpha-smooth muscle actin positive myofibroblasts. Additionally, histological and in-situ hybridization analysis reveal a direct contact of the fracture site with muscle fascia, suggesting a possible involvement of muscle derived cells in the fracture deterioration.

Details

show
hide
Language(s): eng - English
 Dates: 2012-07-312012-10
 Publication Status: Published in print
 Pages: -
 Publishing info: -
 Table of Contents: -
 Rev. Method: Peer
 Identifiers: DOI: 10.1016/j.bone.2012.07.011
 Degree: -

Event

show

Legal Case

show

Project information

show

Source 1

show
hide
Title: Bone
  Other : Bone
Source Genre: Journal
 Creator(s):
Affiliations:
Publ. Info: New York : Elsevier
Pages: - Volume / Issue: 51 (4) Sequence Number: - Start / End Page: 651 - 660 Identifier: ISSN: 8756-3282
CoNE: https://pure.mpg.de/cone/journals/resource/954927629281