Profiling the mitochondrial proteome of Leber's Hereditary Optic Neuropathy (LHON) in Thailand: down-regulation of bioenergetics and mitochondrial protein quality control pathways in fibroblasts with the 11778G>A mutation.

Leber's Hereditary Optic Neuropathy (LHON) is one of the commonest mitochondrial diseases. It causes total blindness, and predominantly affects young males. For the disease to develop, it is necessary for an individual to carry one of the primary mtDNA mutations 11778G>A, 14484T>C or 3460...

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Main Authors: Aung Win Tun, Sakdithep Chaiyarit, Supannee Kaewsutthi, Wanphen Katanyoo, Wanicha Chuenkongkaew, Masayoshi Kuwano, Takeshi Tomonaga, Chayanon Peerapittayamongkol, Visith Thongboonkerd, Patcharee Lertrit
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2014-01-01
Series:PLoS ONE
Online Access:http://europepmc.org/articles/PMC4162555?pdf=render