Maternally inherited diabetes and deafness (MIDD) or mitochondrial diabetes is a multi-system disorder that follows matrilineal inheritance caused by pathogenic variants in the mitochondrial DNA (mtDNA) thus leading to mitochondrial dysfunction. The clinical picture can be variable and mainly depends on the degree of admixture between wild type and mutated mtDNA alleles in an individual (heteroplasmy). It is believed that some patients may be asymptomatic if they do not meet the heteroplasmic threshold, whereas others may exhibit atypical diabetes with progressive symmetrical sensorineural hearing impairment or additional manifestations from the brain, heart, kidneys, retina, peripheral muscles or skeletal nerves [1, 2]. The most common pathogenic variant identified is the A to G transition at position 3243 within the MT-TL1 gene encoding the mitochondrial tRNALeu(UUR), [NC_012920.1(MT-TL1):m.3243 A > G]. Diabetes in MIDD is believed to be caused by a gradual decrease in insulin production due to reduced manufacturing of ATP by the pancreatic cells
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