The principal aim of the proposed STREP is to determine the molecular mechanisms that underpin the pathophysiology of a number of distinct chondrodysplasia phenotypes, which result from mutations in one of a group of different gene products that are important for normal bone development. To achieve this objective we have developed a series of mouse models of chondrodysplasia that closely mimic the relevant human phenotype. We will now study in-depth the growth plate of these mice to determine the molecular, cell and extracellular matrix pathology of these distinct phenotypes.
In this context the objectives of this project are:-
By the end of the project this multidisciplinary systems biology approach will allow us to generate a virtual representation of a cartilage growth plate in health and disease and pave the way for developing therapeutic strategies that might be targeted to a range of individual phenotypes.