Diabetes mellitus is a common disease with many forms of clinical expr
ession. In addition, the development of diabetic complications is not
only dependent on glycaemic control but also on individual factors whi
ch may be related to genetic heterogeneity. At present, multiple genet
ic factors are being recognized as contributing to the development of
diabetes or possibly modulating its clinical expression. The purpose o
f this review is to give an overview of our current knowledge on a sub
type of diabetes which is apparently caused by a single mutation in th
e mitochondrial DNA.