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Optical Coherence Tomography: Imaging Mouse Retinal Ganglion Cells In Vivo
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Mitochondrial optic neuropathies.

Valerio Carelli1, Chiara La Morgia1, Patrick Yu-Wai-Man2

  • 1Department of Biomedical and Neuromotor Sciences, University of Bologna, Bologna, Italy; IRCCS Istituto di Scienze Neurologiche di Bologna, Programma di Neurogenetica, Bologna, Italy.

Handbook of Clinical Neurology
|February 22, 2023
PubMed
Summary
This summary is machine-generated.

Mitochondrial optic neuropathies, like Leber

Keywords:
Complex IDOAGene therapyIdebenoneLHONMitochondriaMitochondrial DNAMitochondrial dynamicsMitochondrial fissionMitochondrial fusionMitochondrial optic neuropathiesOPA1Optic atrophyOptic nerveRetinal ganglion cellsmtDNA

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Area of Science:

  • Mitochondrial medicine and neurogenetics.
  • Ophthalmology and optic nerve disorders.
  • Cellular biology and mitochondrial function.

Background:

  • Mitochondrial optic neuropathies are a significant group of inherited optic nerve disorders.
  • Leber's hereditary optic neuropathy (LHON) and OPA1-related autosomal dominant optic atrophy (DOA) are key examples, impacting retinal ganglion cells (RGCs).
  • These conditions arise from mitochondrial dysfunction, specifically affecting respiratory complex I (LHON) or mitochondrial dynamics (DOA).

Conclusions:

  • Mitochondrial optic neuropathies represent a spectrum of diseases driven by mitochondrial dysfunction.
  • Understanding the specific genetic and molecular basis is crucial for diagnosis and management.
  • Therapeutic interventions, including gene therapy and drugs like idebenone, are under active investigation.