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Conclusion TRPS1 has an important function in regulating bone and dentin mineralization. Having large dental pulp spaces suggests that impaired dentin mineralization was the result of the TRPS1 pathogenic variant. This is the first patient with a TRPS1 pathogenic variant who had impaired dentin mineralization. This is also the third report showing the association between TRPS1 pathogenic variants and the presence of supernumerary teeth.Aims In cardiomyocytes, there is microRNA (miR) in the mitochondria that originates from the nuclear