Characteristics of Japanese patients with Leber’s hereditary optic neuropathy and idebenone trial: a prospective, interventional, non-comparative study
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DOI: 10.1007/s10384-020-00789-2
Abstract: Purpose Leber’s hereditary optic neuropathy (LHON) is a mitochondrial neuropathy that causes acute vision loss. Idebenone, a short-chain ubiquinone analog that preserves mitochondrial function is thought to suppress disease progression in early-onset LHON patients. We… read more here.
Keywords: study; hereditary optic; idebenone; leber hereditary ... See more keywords
Topographic Macular Microvascular Changes and Correlation With Visual Loss in Chronic Leber Hereditary Optic Neuropathy.
Sign Up to like & getrecommendations! Published in 2018 at "American journal of ophthalmology"
DOI: 10.1016/j.ajo.2018.05.029
Abstract: PURPOSE To study the macular microvascular networks in patients affected by chronic Leber hereditary optic neuropathy (LHON) using optical coherence tomography angiography (OCTA), and to quantify these changes in different macular sectors. DESIGN Prospective cross-sectional… read more here.
Keywords: macular microvascular; ophthalmology; chronic leber; hereditary optic ... See more keywords
Atypical Leber hereditary optic neuropathy with a 34-year interval between vision loss in both eyes
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DOI: 10.1016/j.ajoc.2022.101263
Abstract: Purpose Leber hereditary optic neuropathy (LHON) is an inherited mitochondrial disease characterized by painless vision loss affecting both eyes. The disease usually develops in both eyes within weeks to months of onset. We report a… read more here.
Keywords: vision loss; eye; hereditary optic; loss ... See more keywords
Renal artery aneurysm associated with Leber hereditary optic neuropathy
Sign Up to like & getrecommendations! Published in 2018 at "Journal of Vascular Surgery Cases and Innovative Techniques"
DOI: 10.1016/j.jvscit.2017.10.001
Abstract: Leber hereditary optic neuropathy is an inherited, rare, mitochondrial metabolic disease that leads to progressive vision loss due to the accumulation of reactive oxygen species. The disorder has been associated with microangiopathy and macroangiopathy. We… read more here.
Keywords: renal artery; artery aneurysm; hereditary optic; leber hereditary ... See more keywords
Whole mitochondrial genome analysis in South Indian patients with Leber's hereditary optic neuropathy.
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DOI: 10.1016/j.mito.2016.10.006
Abstract: Leber's hereditary optic neuropathy (LHON) is a mitochondrial DNA (mtDNA) associated neurodegenerative disorder of retinal ganglion cells. In this study, whole mitochondrial genome sequencing of 75 LHON patients and 40 controls was performed to identify… read more here.
Keywords: whole mitochondrial; south indian; hereditary optic; mitochondrial genome ... See more keywords
Investigating Leber's hereditary optic neuropathy: Cell models and future perspectives.
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DOI: 10.1016/j.mito.2016.11.006
Abstract: Leber's hereditary optic neuropathy (LHON) was the first human disease found to be associated with a mitochondrial DNA (mtDNA) point mutation. The most common LHON mutations are 11778G>A, 3460G>A or 14484T>C. The most common clinical… read more here.
Keywords: leber hereditary; cell models; hereditary optic; optic neuropathy ... See more keywords
Generation of a human iPSC line, FINCBi001-A, carrying a homoplasmic m.G3460A mutation in MT-ND1 associated with Leber's Hereditary optic Neuropathy (LHON).
Sign Up to like & getrecommendations! Published in 2020 at "Stem cell research"
DOI: 10.1016/j.scr.2020.101939
Abstract: Leber's Hereditary Optic Neuropathy (LHON) is a maternally inherited disorder caused by homoplasmic mutations of mitochondrial DNA (mtDNA). LHON is characterized by the selective degeneration of the retinal ganglion cells (RGC). Almost all LHON maternal… read more here.
Keywords: lhon; neuropathy lhon; carrying homoplasmic; hereditary optic ... See more keywords
Dominant Optic Atrophy and Leber's Hereditary Optic Neuropathy: Update on Clinical Features and Current Therapeutic Approaches.
Sign Up to like & getrecommendations! Published in 2017 at "Seminars in pediatric neurology"
DOI: 10.1016/j.spen.2017.06.001
Abstract: Dominant optic atrophy (DOA) and Leber hereditary optic neuropathy (LHON) are the two most common inherited optic neuropathies encountered in clinical practice. This review provides a summary of recent advances in the understanding of the… read more here.
Keywords: dominant optic; optic atrophy; hereditary optic; leber hereditary ... See more keywords
Propagation and Selectivity of Axonal Loss in Leber Hereditary Optic Neuropathy
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DOI: 10.1038/s41598-019-43180-z
Abstract: Leber hereditary optic neuropathy (LHON) is a syndrome of subacute loss of central vision associated with mutations in mitochondrial DNA coding for components of complex I. LHON preferentially involves small axons in the temporal optic… read more here.
Keywords: propagation; leber hereditary; hereditary optic; axonal loss ... See more keywords
Increased Microcirculation on Optic Nerve Head by Laser Speckle Flowgraphy at Early Stage of Leber Hereditary Optic Neuropathy
Sign Up to like & getrecommendations! Published in 2019 at "Neuro-Ophthalmology"
DOI: 10.1080/01658107.2018.1526956
Abstract: ABSTRACT Leber hereditary optic neuropathy (LHON) is a mitochondrial disorder predominantly affecting young men. Characteristic features of an early stage of LHON are peripapillary telangiectatic microangiopathy with optic disc hyperaemia and swelling of the retinal… read more here.
Keywords: hereditary optic; early stage; microcirculation; leber hereditary ... See more keywords
The Natural History of Leber’s Hereditary Optic Neuropathy in an Irish Population and Assessment for Prognostic Biomarkers
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DOI: 10.1080/01658107.2022.2032761
Abstract: ABSTRACT In this study we have assessed the clinical and genetic characteristics of an Irish Leber’s hereditary optic neuropathy (LHON) cohort and assessed for useful biomarkers of visual prognosis. We carried out a retrospective review… read more here.
Keywords: natural history; history leber; optic neuropathy; hereditary optic ... See more keywords