Pathophysiology of the cartliage growh plate

该项目的主要目的是确定软骨发育不良表型的分子、细胞细胞外基质病理学，这些表型是由对正常骨发育重要的各种不同基因产物的突变引起的。为了实现我们的目标，Eurogrow联盟已经开发了一系列广泛的软骨发育不良小鼠模型，这些模型密切模仿相关的人类表型。该联盟将使用多学科系统生物学方法来确定这些不同软骨发育不良的病理生理学基础的分子机制。通过这种方法，我们可以期望确定常见的疾病机制，并了解软骨发育不良表型中基因型-表型相关性的一般原则。NHMRC-EU赠款将资助一个由10个欧盟研究小组（由曼彻斯特大学领导）组成的财团（Eurogrow），沿着我在MCRI的研究小组进行这些综合研究。

The principle objective of the proposed project is to determine the molecular, cellular extracellular matrix pathology of chondrodysplasia phenotypes which result from mutations in a variety of different gene products that are important for normal bone development. To achieve our objective the Eurogrow consortium has developed an extensive series of mouse models of chondrodysplasias that closely mimic the relevant human phenotype. The consortium will use a multidisciplinary systems biology approach to determine the molecular mechanisms that underpin the pathophysiology of these distinct chondrodysplasias. From this approach we can expect to identify common disease mechanisms and learn general principles about genotype-phenotype correlations in chondrodysplasia phenotypes. The NHMRC-EU grant is to fund a consortium (Eurogrow) of 10 EU research groups (led by the University of Manchester) along with my research group at the MCRI to conduct these integrated studies.