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

S Pragnère, J-C Auregan, C Bosser, A Linglart, M Bensidhoum, T Hoc, C Nouguier-Lehon, C Chaussain

Journal: Acta biomaterialia 2021;119():259-267

PMID: 33122145

Full Text on Elsevier Science View on PubMed

Abstract

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

Address: Equipex IVTV, Centrale Innovation, 64 Chemin des Mouilles, 69130 Ecully, France.; Université de Paris, B3OA, UMR CNRS 7052, INSERM U1271, 10 Avenue de Verdun, 75010 Paris, France; AP-HP, Antoine Béclère Université Paris-Saclay hospital, Orthopeadics Department, 157, rue de la Porte de Trivaux, 92140 Clamart, France.; Université de Paris Saclay, Le Kremlin-Bicêtre, France; AP-HP, Department of Endocrinology and Diabetology for children, Reference Center for Rare Disorders of the Calcium and Phosphate Metabolism, Filière OSCAR and Platform of expertise for rare diseases Paris-Sud, Bicêtre Paris-Saclay Hospital, Le Kremlin-Bicêtre, France.; Université de Paris, B3OA, UMR CNRS 7052, INSERM U1271, 10 Avenue de Verdun, 75010 Paris, France.; Université de Paris, B3OA, UMR CNRS 7052, INSERM U1271, 10 Avenue de Verdun, 75010 Paris, France; Mechanical Department, MSGMGC, Ecole Centrale de Lyon, 36 Avenue Guy de Collongue, 69134 Ecully Cedex, France. Electronic address: [email protected].; Université de Lyon, LTDS UMR CNRS 5513, Ecole Centrale de Lyon, 36 Avenue Guy de Collongue, 69134 Ecully Cedex, France.; Université de Paris, Dental School, UR2496, Montrouge, F-92120, France; AP-HP Reference Center for Rare Disorders of the Calcium and Phosphate Metabolism (OSCAR, ERN Bond), Dental Medicine Department, Bretonneau Hospital, GHN, 75018 Paris, France.

DOI: 10.1016/j.actbio.2020.10.033