Fig. 3.

Patients 4 and 5. A, Pedigree. Patient 4 (I-2) is the grandfather of patient 5 (III-1), whose mother has a compound heterozygous mutation c.1039G>T; p.Glu347X and c.673G>A; p.Gly213Ser in WNT10A. Patients 4 and 5 are heterozygous for the c.1039G>T; p.Glu347X variant in WNT10A. B, Panoramic radiograph of patient 4 at age 58 shows taurodontism and pulp stones in the maxillary right first permanent molar and maxillary second permanent molars (asterisks). Severe pulp obliteration is evident in most teeth. C, Panoramic radiograph of patient 5 at age 8 shows mixed dentition, agenesis of the mandibular first left central incisor (arrowhead), and taurodontism of the maxillary first permanent molars (asterisks). Microdontia of the maxillary permanent lateral incisors and the developing maxillary and mandibular right second permanent molars was noted (arrows).