作者: D.S. Brown , B.F. Eames
DOI: 10.1016/BS.MCB.2016.03.001
关键词:
摘要: In the past 20years, appreciation for varied roles of proteoglycans (PGs), which are specific types sugar-coated proteins, has increased dramatically. PGs in extracellular matrix were long known to impart structural functions many tissues, especially articular cartilage, cushions bones and allows mobility at skeletal joints. Indeed, osteoarthritis is a debilitating disease associated with loss cartilage. Today, however, have demonstrated role cell biological processes, such as growth factor signalling, prompting new perspectives on etiology PG-associated diseases. Here, we review diseases defects PG synthesis sulfation, also highlighting current understanding underlying genetics, biochemistry, biology. Since most research analyzed class called heparan sulfate PGs, more attention paid here studies chondroitin (CSPGs), abundant Interestingly, CSPG tightly linked processes secretion lysosomal degradation, suggesting that these systems may be genetically. Animal models function revealed CSPGs impact development. Specifically, our work from mutagenesis screen zebrafish led hypothesis cartilage normally delay timing endochondral ossification. Finally, outline emerging approaches revolutionize study function, including transgenic methods novel imaging techniques. Our recent X-ray fluorescent imaging, example, enables direct correlation PG-dependent processes.