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Dentinogenesis imperfecta (DGI) is characterized by discolored teeth with an opalescent sheen and dentin that fails to support enamel, causing it to easily chip. Two new studies show that DGI is associated with mutations in DSPP, a gene encoding dentin sialophosphoprotein that is processed into two proteins: dentin sialoprotein (DSP) and dentin phosphoprotein (DPP). These are key components of the dentin extracellular matrix (DECM). Notably, missense mutations in DSPP are also associated with progressive hearing loss.

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Figure 1: Tooth development during the apposition phase.

Bob Crimi

Figure 2: Schematic diagram of the dentin sialophosphoprotein gene (DSPP) (a), mRNA (b) and protein products (c).

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  1. Departments of Neurology, and Molecular and Human Genetics, Baylor College of Medicine, Houston, Texas

    Pragna I Patel

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  1. Pragna I Patel
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Patel, P. Soundbites. Nat Genet 27, 129–130 (2001). https://doi.org/10.1038/84728

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  • DOI: https://doi.org/10.1038/84728

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