Diabetic symptoms caused by or involving mitochondrial dysfunction are generally associated with various complications, especially neurological ones, such as MELAS, KSS and LHON.

Other clinical features accompanying diabetes are primarily dependent on mtDNA or nuclear DNA mutations. This association has been valued in families with massive mtDNA rearrangements (replications) or m.3243A>G mtDNA point mutations. Subsequently, maternally inherited diabetes and deafness (MIDD) was recognized as a distinct subtype of diabetes associated with the m.3243A>G mutation.

Mitochondrial dysfunction can lead to islet beta cell dysfunction and insulin resistance in other parts of the body. Therefore, mitochondria are of special concern in both type I and type II diabetes.