[A new subtype of diabetes mellitus: maternally inherited diabetes and deafness (MIDD)]

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J A Maassen1, J J Jansen, J M van den Ouweland

  • 1Rijksuniversiteit, vakgroep Medische Biochemie, Leiden.

Nederlands Tijdschrift Voor Geneeskunde
|April 29, 1998
PubMed

Diabetes mellitus comprises many subtypes, the pathogenesis of each of which involves a combination of inherited and environmental factors. Recently a new subtype of diabetes mellitus was recognized in a Dutch pedigree, designated as 'maternally inherited diabetes and deafness' (MIDD). Impaired hearing is an associated phenomenon of the disease. Approximately 1.3% of all diabetic cases in the Netherlands exhibit the MIDD subtype. MIDD shows a strictly maternal heredity. In MIDD there is a guanine-for-adenine substitution at position 3243 in mitochondrial DNA. Mitochondria carrying this mutation exhibit a decreased functionality. In carriers of the MIDD mutation the insulin secretion by the pancreas in response to stimulation by glucose is impaired.

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