MITOCHONDRIAL DYSFUNCTION IN IDIOPATHIC PARKINSON-DISEASE

Disordered mitochondrial metabolism may play an important role in a nu mber of idiopathic neurodegenerative disorders. The question of mitoch ondrial dysfunction is particularly attractive in the case of idiopath ic Parkinson disease (PD), since Vyas et al. recognized in the 1980s t hat the parkinsonism-inducing compound N-methyl-4-phenyl-1,2,3,6-tetra hydropyridine is a mitochondrial toxin. The unique genetic properties of mitochondria also make them worthy of consideration for a pathogeni c role in PD, as well as in other late-onset, sporadic neurodegenerati ve disorders. Although affected persons occasionally do provide family histories that suggest Mendelian inheritance, the vast majority of th e time these diseases appear sporadically. Because of unique features such as heteroplasmy, replicative segregation, and threshold effects, mitochondrial inheritance can allow for the apparent sporadic nature o f these diseases.