Mitochondrial DNA is inherited maternally and encodes several genes in the oxidative phosphorylation pathway, ribosomal RNAs, and 22 transfer RNAs (tRNAs).

In rare cases, (<1%), diabetes is associated with point mutations in a mitochondrial tRNA gene, tRNALeu(UUR).91 Mitochondrial diabetes is caused by a primary defect in ?-cell function.

Recall that ATP is required for insulin secretion in ? cells, and impairment of mitochondrial ATP synthesis results in decreased insulin secretion.

References

 Niaudet P, Rotig A. Renal involvement in mitochondrial cytopathies. Pediatr Nephrol. 1996 Jun;10(3):368-73. PMID: #8792408#