Care4BrittleBones aims to improve the quality of life of people with Brittle Bones Disease

Latest scientific insights: Research Brief “Why are the bones so fragile”?

Updates on OI research

Based on a presentation to the Dutch OI Association VOI Prof.dr. R A. Bank, University Medical Center Groningen (NL) has worked with Care4BrittleBones to summarize his findings about a topic that interests most people with OI: “Why are the bones actually so fragile”? The research was conducted in collaboration with Dr. R. Sakkers, Wilhelmina Children’s Hospital (NL).

Is it the amount of mineralised collagen or damaged (broken) collagen? Is it the placement of the collagen in the fibrils of the bone matrix or the cross-links in between? Interestingly, the answer to these questions was not really known, until Prof. Bank/Dr. Sakkers brought some light into this area. The answer is: none of the above causes the brittleness! Instead it is due to:
• Slight decrease in collagen content (hypermineralization)
• Great increase of the amount of hydroxylysine in collagen
• Great increase of glycosylated hydroxylysine
As a consequence: There is more mineral between the collagen molecules (hypermineralization) and fibrils (bundles of collagen fibers) therefore are thinner (inverse relationship).

Still hard to understand? Download here the full article, which gives you detailed insight on what’s happening in the bone.