Human dentin characteristics of patients with osteogenesis imperfecta: insights into collagen-based biomaterials

Osteogenesis imperfecta (OI), also known as "brittle bone disease", is a rare genetic disorder of the skeleton, whose most benign form I corresponds to autosomal dominant mutations in genes encoding type collagen (COLA1, COLA2). Several associated skeletal manifestations are often observed but, surprisingly, while dentin defects reflect disorders, about half OI patients have no obvious oral manifestations. Here, we investigated collagen, mineral and mechanical properties from deciduous teeth collected with mild displaying clinical signs dentinogenesis imperfecta. For first time, an increase hardness content compared healthy was reported. In addition, altered tissue characteristics dentin-enamel junction but interfacial gradient preserved. The impact changes molecular structure due assessed by Raman microspectroscopy. Our results highlighted change hydroxyproline-proline ratio direct association mineralization. findings suggest that evaluation could be important aid for types difficult diagnose clinically provide experimental evidence hydroxyproline should considered future studies on collagen-based biomaterials.