THE ROLE OF A NOVEL HYALURONIDASE IN THE TURNOVER OF CHONDROITIN SULPHATE GLYCOSAMINOGLYCANS IN CARTILAGE AND BONE

复杂碳水化合物结构的降解发生在专门的细胞内细胞器(溶酶体)内。它们的降解发生在严格定义的序列中，包括最初剪切成中等大小的片段，然后从一端逐个片段地降解。在这个提案中，我们试图了解一种新定义的酶在最初的剪裁过程中的作用。这些信息将有助于我们理解复杂碳水化合物降解受损的一组遗传疾病的骨骼病理。此外，由于复杂碳水化合物的广泛分布及其在组织发育和生长中的基本作用，该项目对癌症和伤口修复等不同疾病状态具有更广泛的影响。

The degradation of complex carbohydrate structures occurs within specialised intracellular organelles (lysosomes). Their degradation occurs in a strictly defined sequence involving initial clipping into intermediate sized fragments which are each then degraded piece by piece from one end. In this proposal we seek to understand the role of a newly defined enzyme in the initial clipping process. This information will aid our understanding of skeletal pathology in a group of genetic disorders in which complex carbohydrate degradation is impaired. In addition due to the widespread location of complex carbohydrates and their fundamental roles in tissue development and growth, the project has wider implications in diverse disease states such as cancer and wound repair.