Mitochondrial neurogastroinstestinal encephalomyopathy (MNGIE – OMIM# 603041) is a rare multisystem autosomal recessive disorder caused by homozygous or compound heterozygous mutations in the nuclear-encoded thymidine phosphorylase gene (TYMP; 131,222) on chromosome 22q13, the first gene whose role was defined at molecular level in the defects of intergenomic communication [1]. The gene discussed is TYMP; the disease is mitochondrial neurogastrointestinal encephalomyopathy.