Abstract

While traditionally the function of the extracellular matrix and the basement membrane was considered to be providing structural support, it is now clear that this only covers one aspect of its multiple functions. This is also illustrated by our growing knowledge of the role of collagen IV, a major component of basement membranes, in development, health, and disease. With the extracellular matrix and collagen IV increasingly being recognised as key players in a growing number of diseases from stroke and vascular defects to kidney disease, deafness, and eye abnormalities, it is paramount that we increase our fundamental understanding of these complex molecules ranging from their biosynthesis to their role in human disease. Recently, exciting progress has been made in delineating the mechanisms by which mutations in collagen IV cause disease, and these are being exploited to develop mechanism-based treatments. Yet many important questions remain that need addressing to develop treatments for diseases associated with collagen IV.