MATERNAL INHERITANCE AND THE EVALUATION OF OXIDATIVE-PHOSPHORYLATION DISEASES

Mitochondrial DNA is more susceptible than nuclear DNA to mutations. M itochondrial mutations have been associated with a range of disorders, some of which can be inherited maternally as well as by mendelian pat terns. The oxidative phosphorylation diseases are a group of such diso rders characterised by a complex phenotype; the Kearns-Sayre syndrome, for example, can include cardiac abnormalities, diabetes mellitus, ce rebellar ataxia, and deafness. An understanding of the genetic and bio chemical basis of these disorders wilt help in the adoption of a syste matic approach to their diagnosis and to patient management.