Abstract
Modelling neurofibromatosis type 1 tibial dysplasia and its treatment with lovastatin
Highlights
Bowing and/or pseudarthrosis of the tibia is a known severe complication of neurofibromatosis type 1 (NF1)
Our data suggest the potential usefulness of lovastatin, a drug approved by the US Food and Drug Administration in 1987 for the treatment of hypercholesteraemia, in the treatment of Nf1-related fracture healing abnormalities
The experimental model presented here constitutes a valuable tool for the pre-clinical stage testing of candidate drugs, targeting Nf1-associated bone dysplasia
Summary
Bowing and/or pseudarthrosis of the tibia is a known severe complication of neurofibromatosis type 1 (NF1). We studied bone healing in a cortical bone injury model in Nf1Prx mice as a model for NF1associated bone disease. Taking advantage of this experimental model we explore effects of systemically applied lovastatin, a cholesterol-lowering drug, on the Nf1 deficient bone repair. Usually of the tibia, is a well known and serious complication of neurofibromatosis type 1 (NF1) [1,2,3]. Therapeutic programs have been largely based on conceptual considerations for the treatment of post-traumatic nonunions These forms of treatment, are often futile when applied to pseudarthrosis of the tibia indicating that systemic problems interfere with normal healing.
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