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Institut Necker professor Valérie Cormier-Daire is heading a research project to improve the treatment of osteogenesis imperfecta (OI). This condition also known as brittle bone disease is one of the most frequent congenital bone disorders, with an estimated occurrence of one in every 15,000 births. It is characterized by low bone density which causes repeated fracturing occurring after mild traumas.
Osteogenesis imperfecta has widely differing clinical and molecular forms. The most severe cases develop in the womb whereas more benign forms appear only in adulthood. Whatever its nature, the condition is linked to genetic disorders affecting the production of type 1 collagen, a protein found in connective tissues and more particularly in the composition of the bones, skin, teeth and ligaments. In the case of the bones, the fragility of the bone tissue observed in OI is caused by alterations in the bone matrix, 25% of which consists of type 1 collagen secreted by cells known as osteoblasts.
The most frequent treatment, which is offered to children and to the severest cases, is effective but draconian, resulting over a long period in a modification to the structure and quality of the bone. In addition, it may prove ineffective for some recessive forms of OI. To overcome this, the Institut Necker is carrying out an ambitious research project, looking into the development of a treatment targeting osteoblasts to produce better quality bone tissue. The Michelin Corporate Foundation is backing this promising project.