Mutations of DLX3 are closely related to Tricho-Dento-Osseous syndrome (TDO; OMIM 190320), which is a disorder with autosomal dominant inheritance mainly characterized by kinky hair, thin-pitted enamel (with remarkable attrition), dentin hypoplasia, and taurodontism as well as increased thickness and density of cranial and mandibular bones. This evidence concerns the gene DLX3 and tricho-dento-osseous syndrome.