The observation that, similar to the mouse Dio3, the human DIO3 gene is imprinted and preferentially expressed from the paternally inherited allele (65) also supports a role for DIO3 deficiency and subsequent TH excess in the etiology of Temple syndrome, a condition that develops as a result of maternal uniparental disomy of chromosome 14 (66, 67). The gene discussed is DIO3; the disease is motor developmental delay due to 14q32.2 paternally expressed gene defect.