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Mitochondrial dysfunction in idiopathic Parkinson disease.

Abstract：

:Disordered mitochondrial metabolism may play an important role in a number of idiopathic neurodegenerative disorders. The question of mitochondrial dysfunction is particularly attractive in the case of idiopathic Parkinson disease (PD), since Vyas et al. recognized in the 1980s that the parkinsonism-inducing compound N-methyl-4-phenyl-1,2,3,6-tetrahydropyridine is a mitochondrial toxin. The unique genetic properties of mitochondria also make them worthy of consideration for a pathogenic role in PD, as well as in other late-onset, sporadic neurodegenerative disorders. Although affected persons occasionally do provide family histories that suggest Mendelian inheritance, the vast majority of the 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 of these diseases.

journal_name

Am J Hum Genet

journal_title

American journal of human genetics

authors

Parker WD Jr,Swerdlow RH

doi

10.1086/301812

subject

Has Abstract

pub_date

1998-04-01 00:00:00

pages

758-62

issue

4

eissn

0002-9297

issn

1537-6605

pii

S0002-9297(07)60964-1

journal_volume

62

pub_type

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