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Mitochondrial diabetes

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A subtype of diabetes and also a type of mitochondrial disease characterized by hearing loss and hyperglycemia (due to deficiency of the hormone insulin)


Diabetes is a condition characterized by excessive glucose level in the blood and urine and mitochondrial diabetes is a subtype. The individual with this type of diabetes has low levels of insulin. The insulin is a hormone that regulates the amount of glucose in the bloodstream. In mitochondrial diabetes, the underlying cause is mutation in the MT-TL1, MT-TK, or MT-TE gene. These genes are located in the mitochondrial DNA. Pathological mutation involving any of these genes in the pancreas ultimately results in dysfunctional mitochondria. Normally, mitochondria help trigger the release of insulin during high blood glucose levels. As such, mitochondrial diabetes is also a type of a mitochondrial disease.

Mitochondrial diabetes is inherited in a mitochondrial pattern. This means that the individual with this condition inherited the disorder from their carrier mother. This form of inheritance is referred to as maternal inheritance. This is because the egg cell is the source of the mitochondrial DNA.


  • diabetes mellitus type II with deafness
  • Ballinger-Wallace syndrome
  • diabetes mellitus and deafness
  • maternally inherited diabetes and deafness
  • diabetes-deafness syndrome

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