Background: Zinc (Zn) is an essential trace element and it is abundant in connective tissues, however biological roles of Zn and its transporters in those tissues and cells remain unknown.

Background: Zinc (Zn) is an essential trace element and it is abundant in connective tissues, however biological roles of Zn and its transporters in those tissues and cells remain unknown.Methodology/Principal Findings: Here we report that mice deficient in Zn transporter Slc39a13/Zip13 show changes in bone, teeth and connective tissue reminiscent of the clinical spectrum of human Ehlers-Danlos syndrome (EDS). The Slc39a13 knockout (Slc39a13-KO) mice show defects in the maturation of osteoblasts, chondrocytes, odontoblasts, and fibroblasts. In the corresponding tissues and cells, impairment in bone morphogenic protein (BMP) and TGF-b signaling were observed. Homozygosity for a SLC39A13 loss of function mutation was detected in sibs affected by a unique variant of EDS that recapitulates the phenotype observed in Slc39a13-KO mice.Conclusions/Significance: Hence, our results reveal a crucial role of SLC39A13/ZIP13 in connective tissue development at least in part due to its involvement in the BMP/TGF-b signaling pathways. The Slc39a13-KO mouse represents a novel animal model linking zinc metabolism, BMP/TGF-b signaling and connective tissue dysfunction.

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Some specific factors in this patient population affect the dental caries and tooth extraction and oral programs targeting these factors may reduce dental degeneration in these patients.

Unicystic ameloblastoma (UAM) is a variant of intraosseous ameloblastoma that occurs as a single cystic cavity. This report describes a case of UAM of the mandible in a seven-year-old girl. The lesion radiographically mimicked a dentigerous cyst. Under the primary diagnosis of a dentigerous cyst, marsupialization was performed to erupt the first molar involved in the cystic lesion and to obtain a definitive diagnosis. The biopsy specimen revealed ameloblastoma. During careful observation, orthodontic treatment, which was performed to upright and promote the eruption of the first molar involved in the tumor, maintained the space needed for enucleation of the tumor. Finally, the second primary molar was extracted, and the lesion was enucleated at 3 years and 4 months after marsupialization. The results of the histological examination revealed UAM. Conclusively, the treatment course not only avoids a resection of the mandible but also induces eruption of the teeth involved in the tumor. Thus, the combination of conservative surgery and orthodontic treatment was effective in the management of UAM that mimics a dentigerous cyst.