Raised intraocular pressure as a potential risk factor for visual loss in Leber Hereditary Optic Neuropathy.

Leber Hereditary Optic Neuropathy (LHON) is an important cause of inherited mitochondrial blindness among young adults. The majority of patients carry one of three mitochondrial DNA (mtDNA) point mutations: m.3460G>A, m.11778G>A and m.14484T>C, all of which affect critical complex I subunit...

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Main Authors: Anais Thouin, Philip G Griffiths, Gavin Hudson, Patrick F Chinnery, Patrick Yu-Wai-Man
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2013-01-01
Series:PLoS ONE
Online Access:https://journals.plos.org/plosone/article/file?id=10.1371/journal.pone.0063446&type=printable
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author Anais Thouin
Philip G Griffiths
Gavin Hudson
Patrick F Chinnery
Patrick Yu-Wai-Man
author_facet Anais Thouin
Philip G Griffiths
Gavin Hudson
Patrick F Chinnery
Patrick Yu-Wai-Man
author_sort Anais Thouin
collection DOAJ
description Leber Hereditary Optic Neuropathy (LHON) is an important cause of inherited mitochondrial blindness among young adults. The majority of patients carry one of three mitochondrial DNA (mtDNA) point mutations: m.3460G>A, m.11778G>A and m.14484T>C, all of which affect critical complex I subunits of the mitochondrial respiratory chain. LHON is characterised by marked incomplete penetrance, clearly implying that the mtDNA mutation is insufficient on its own to trigger retinal ganglion cell dysfunction and visual loss. In this case series of three affected patients harbouring the m.11778G>A mutation, we provide evidence suggesting that raised intraocular pressure could be a risk factor triggering visual loss in at-risk LHON carriers.
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institution OA Journals
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language English
publishDate 2013-01-01
publisher Public Library of Science (PLoS)
record_format Article
series PLoS ONE
spelling doaj-art-3f4ad905d9974aec8542e17a78b892d22025-08-20T02:30:37ZengPublic Library of Science (PLoS)PLoS ONE1932-62032013-01-0185e6344610.1371/journal.pone.0063446Raised intraocular pressure as a potential risk factor for visual loss in Leber Hereditary Optic Neuropathy.Anais ThouinPhilip G GriffithsGavin HudsonPatrick F ChinneryPatrick Yu-Wai-ManLeber Hereditary Optic Neuropathy (LHON) is an important cause of inherited mitochondrial blindness among young adults. The majority of patients carry one of three mitochondrial DNA (mtDNA) point mutations: m.3460G>A, m.11778G>A and m.14484T>C, all of which affect critical complex I subunits of the mitochondrial respiratory chain. LHON is characterised by marked incomplete penetrance, clearly implying that the mtDNA mutation is insufficient on its own to trigger retinal ganglion cell dysfunction and visual loss. In this case series of three affected patients harbouring the m.11778G>A mutation, we provide evidence suggesting that raised intraocular pressure could be a risk factor triggering visual loss in at-risk LHON carriers.https://journals.plos.org/plosone/article/file?id=10.1371/journal.pone.0063446&type=printable
spellingShingle Anais Thouin
Philip G Griffiths
Gavin Hudson
Patrick F Chinnery
Patrick Yu-Wai-Man
Raised intraocular pressure as a potential risk factor for visual loss in Leber Hereditary Optic Neuropathy.
PLoS ONE
title Raised intraocular pressure as a potential risk factor for visual loss in Leber Hereditary Optic Neuropathy.
title_full Raised intraocular pressure as a potential risk factor for visual loss in Leber Hereditary Optic Neuropathy.
title_fullStr Raised intraocular pressure as a potential risk factor for visual loss in Leber Hereditary Optic Neuropathy.
title_full_unstemmed Raised intraocular pressure as a potential risk factor for visual loss in Leber Hereditary Optic Neuropathy.
title_short Raised intraocular pressure as a potential risk factor for visual loss in Leber Hereditary Optic Neuropathy.
title_sort raised intraocular pressure as a potential risk factor for visual loss in leber hereditary optic neuropathy
url https://journals.plos.org/plosone/article/file?id=10.1371/journal.pone.0063446&type=printable
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