Subclinical thinning of macular ganglion cell layer in Leber optic neuropathy carriers

González-Martín-Moro, J.; Castro-Rebollo, M.; Contreras, Inés; Pérez-Sarriegui, Ane

doi:10.1111/cxo.12634

Cited by 3 publications

(2 citation statements)

References 13 publications

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“…Dël ðios dis funk ci jos tin klai nës gan gli në se làs telë se su ma þë ja ATP sin te zë ir pa di dë ja ok si da ci nio stre so ly gis. Tai le mia làs te liø de ge ne ra ci jà ir apop to zae [7][8][9][10]. PLON daþ nis svy ruo ja nuo 1:31 000 iki 1:54 000 at ve jø per me tus.…”

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Treatment options for Leber hereditary optic neuropathy

Povilaitytė¹,

Liutkevičienė²

2018

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Paveldima Lėberio optinė neuropatija (PLON) yra dažniausiai pasireiškianti paveldima optinė neuropatija, sukelta pirminių mutacijų mitochondrinėje DNR. PLON pasireiškia regos nervo atrofija ir centrinio matymo praradimu. Apie 70 % PLON atvejų nustatomos trys pagrindinės mutacijos mitochondrinėje DNR (mtDNR): 11778G>A/ND4, 3460G>A/ND1 ir 14484T>C/ND6. Taip pat aprašyta daugiau nei 18 novelinių mtDNR mutacijų, siejamų su PLON ir lemiančių mitochondrijų elektronų transporto grandinės disfunkciją. Dėl šios disfunkcijos tinklainės ganglinėse ląstelėse sumažėja ATF sintezė ir padidėja oksidacinio streso lygis. Tai lemia ląstelių degeneraciją ir apoptozę. Šiame straipsnyje apžvelgiame pagrindines paveldimos Lėberio optinės neuropatijos pasireiškimo išraiškas ir gydymo galimybes.

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Treatment options for Leber hereditary optic neuropathy

Povilaitytė¹,

Liutkevičienė²

2018

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“…Risk factors for phenotypic manifestation of LHON include smoking and heavy drinking, with penetrance increasing to 93% in male smokers (284). LHON is primarily mediated by oxidative stress and degeneration of RGCs particularly within the papillomacular bundle, which are highly sensitive to dysfunctions in ATP synthesis and mitochondrial malfunction (285,286).…”

Section: 103(a) Leber Hereditary Optic Neuropathymentioning

confidence: 99%

The Impact of Optic Nerve Disorders on Sleep Wake

Stevenson

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Current and Emerging Treatment Modalities for Leber’s Hereditary Optic Neuropathy: A Review of the Literature

et al. 2018

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IntroductionThe purpose of this review is to present the current and emerging treatment alternatives for Leber’s hereditary optic neuropathy (LHON), emphasizing the most recent use of idebenone and stem cells or gene therapy.MethodsA comprehensive literature review was performed at the PubMed database regarding the various treatment modalities for LHON.ResultsTreatment modalities for LHON include nutritional supplements, activators of mitochondrial biogenesis, brimonidine, and symptomatic and supportive treatment, but nowadays attention is being paid to idebenone and gene therapy or stem cells.ConclusionThe treatment of LHON remains challenging, given the nature of the disease and its prognosis.

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Subclinical thinning of macular ganglion cell layer in Leber optic neuropathy carriers

Cited by 3 publications

References 13 publications

Treatment options for Leber hereditary optic neuropathy

Treatment options for Leber hereditary optic neuropathy

The Impact of Optic Nerve Disorders on Sleep Wake

Current and Emerging Treatment Modalities for Leber’s Hereditary Optic Neuropathy: A Review of the Literature

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