Letter to the Editor: Variable response to Idebenone in LHON is multifactorial

Finsterer, Josef; Mehri, Sounira

doi:10.47162/rjme.64.1.13

Rom J Morphol Embryol

2023

DOI: 10.47162/rjme.64.1.13

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Letter to the Editor: Variable response to Idebenone in LHON is multifactorial

Josef Finsterer¹,

Sounira Mehri²

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2023

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“…Dear Editor, We read with interest the letter [ 1 ] from Josef Finsterer & Sounira Mehri about our article regarding the morphological and functional response to Idebenone therapy in patients with Leber hereditary optic neuropathy (LHON), where two pediatric patients, genetically confirmed, were periodically followed-up over a period of one year after we initiated the treatment [ 2 ]. We thank them for the interest shown in this topic and we want to answer the questions they have formulated regarding our study.…”

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Letter to the Editor: Retinal morphological and functional response to Idebenone therapy in Leber hereditary optic neuropathy

Mercuţ,

Tănasie,

Nicolcescu

et al. 2023

Rom J Morphol Embryol

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confidence: 99%

Letter to the Editor: Retinal morphological and functional response to Idebenone therapy in Leber hereditary optic neuropathy

Mercuţ,

Tănasie,

Nicolcescu

et al. 2023

Rom J Morphol Embryol

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Anti-inflammatory reprogramming of microglia cells by metabolic modulators to counteract neurodegeneration; a new role for Ranolazine

Piano,

Votta,

Colucci

et al. 2023

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Microglia chronic activation is a hallmark of several neurodegenerative diseases, including the retinal ones, possibly contributing to their etiopathogenesis. However, some microglia sub-populations have anti-inflammatory and neuroprotective functions, thus making arduous deciphering the role of these cells in neurodegeneration. Since it has been proposed that functionally different microglia subsets also rely on different metabolic routes, we hypothesized that modulating microglia metabolism might be a tool to enhance their anti-inflammatory features. This would have a preventive and therapeutic potential in counteracting neurodegenerative diseases. For this purpose, we tested various molecules known to act on cell metabolism, and we revealed the anti-inflammatory effect of the FDA-approved piperazine derivative Ranolazine on microglia cells, while confirming the one of the flavonoids Quercetin and Naringenin, both in vitro and in vivo. We also demonstrated the synergistic anti-inflammatory effect of Quercetin and Idebenone, and the ability of Ranolazine, Quercetin and Naringenin to counteract the neurotoxic effect of LPS-activated microglia on 661W neuronal cells. Overall, these data suggest that using the selected molecules -also in combination therapies- might represent a valuable approach to reduce inflammation and neurodegeneration while avoiding long term side effects of corticosteroids.

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Letter to the Editor: Variable response to Idebenone in LHON is multifactorial

Cited by 2 publications

References 1 publication

Letter to the Editor: Retinal morphological and functional response to Idebenone therapy in Leber hereditary optic neuropathy

Letter to the Editor: Retinal morphological and functional response to Idebenone therapy in Leber hereditary optic neuropathy

Anti-inflammatory reprogramming of microglia cells by metabolic modulators to counteract neurodegeneration; a new role for Ranolazine

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