Dentinogenesis imperfecta: evidence of qualitative alteration in the organic dentin matrix

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Abstract
Deciduous teeth affected by dentinogenesis imperfecta [an autosomal dominant disorder] were obtained from 2 patients with osteogenesis imperfecta. EM of the dentin revealed some structural alterations. The striation of the collagen fibrils was not clear and the crystals were less dense than in normal dentin. The amino acid analysis of dentin collagen was slightly different from normal dentin. There were slight increases in the amounts of hydroxylysine, serine and acidic acids. On the other hand, lysine and arginine were reduced. The elevation of hexosyllysine content and an increase of carbohydrate were also observed. Dentin collagen is apparently altered, or noncollagenous matrices associated with collagen are apparently increased in dentinogenesis imperfecta dentin.