Matrix Biology Research Articles

Elastases: Structure, Function and Pathological Role, Vol 6: Frontiers of Matrix Biology (Book)

Elastases: Structure, Function and Pathological Role, Vol 6: Frontiers of Matrix Biology (Book)

For the Bicentennial: Historical Marker Commemorates Lititz Pharmacopeia

This issue of Matrix Biology commemorates the memory of Dick Heinegård and his exceptional contributions to identify extracellular matrix molecules and their interactions that form cartilage matrices. This tribute to him demonstrates the development of his cartilage matrix model and how this model relates to the articles in this Matrix Biology Cartilage issue.

DR. Eduardo Perroncito

Osteogenesis imperfecta, or brittle bone disease, is a congenital disease that primarily causes low bone mass and bone fractures but it can negatively affect other organs. It is usually inherited in an autosomal dominant fashion, although rarer recessive and X-chromosome-linked forms of the disease have been identified. In addition to type I collagen, mutations in a number of other genes, often involved in type I collagen synthesis or in the differentiation and function of osteoblasts, have been identified in the last several years. Seldom, the study of a rare disease has delivered such a wealth of new information that have helped our understanding of multiple processes involved in collagen synthesis and bone formation. In this short review I will describe the clinical features and the molecular genetics of the disease, but then focus on how OI dysregulates all aspects of extracellular matrix biology. I will conclude with a discussion about OI therapeutics.