Molecular mechanisms of collagen matrix assembly in health and disease

该项目研究组织的特定蛋白质构建块如何相互作用，以形成组织生长，发育和功能所需的复杂结构网络。具体来说，我们将研究胶原蛋白，身体的主要结构蛋白。我们将确定它们如何与其他蛋白质组装形成复杂的原纤维或分子绳，为组织提供机械强度，并为细胞在骨骼，皮肤，软骨和肌肉等组织中生长和发育创造环境。我们将探讨发生在软骨和肌肉遗传性疾病患者中的胶原突变对这些组装过程的影响。这将提供有关疾病过程的关键信息，以便在未来开发新的诊断和治疗方法。我们从这些研究中获得的对组织结构组装的分子基础的理解对于理解正常组织的生长和发育以及用于治疗用途的新生物材料的开发将是重要的。

This project studies the way the specific protein building blocks of tissues interact with each other to form the complicated structural networks necessary for tissue growth, development and function. Specifically, we will study collagens, the main structural proteins of the body. We will determine how they assemble with other proteins to form the complex fibrils or molecular ropes, which provide mechanical strength to tissues and create the environment for cells to grow and develop in tissues such as bone, skin, cartilage and muscle. We will explore the effect of collagen mutations, that occur in patients with inherited diseases of cartilage and muscle, on these assembly processes. This will provide key information on the disease process, so that in the future new approaches to diagnosis and therapy can be developed. The understanding we gain on the molecular basis of tissue structural assembly from these studies will be of importance for understanding normal tissue growth and development, and for the development of new biomaterials for therapeutic use.