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Determination of Mitochondrial Respiration and Glycolysis in Ex Vivo Retinal Tissue Samples
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Mitochondrial Retinopathies.

Massimo Zeviani1,2, Valerio Carelli3,4

  • 1Department of Neurosciences, The Clinical School, University of Padova, 35128 Padova, Italy.

International Journal of Molecular Sciences
|January 11, 2022
PubMed
Summary
This summary is machine-generated.

Mitochondrial dysfunction in the retina causes vision loss through retinal dystrophy and optic atrophy. Genetic mutations in mitochondrial DNA (mtDNA) or nuclear genes are the primary causes of these conditions.

Keywords:
Kearns-Sayre syndromeLeber’s hereditary optic neuropathy (LHON)ataxia and retinitis pigmentosa (NARP)autosomal dominant optic atrophy (ADOA)mitochondrial DNAmitochondrial disordersmtDNA heteroplasmic deletionsneurogenic muscle weaknessoptic atrophyretinaretinitis pigmentosa

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Area of Science:

  • Ophthalmology
  • Genetics
  • Mitochondrial Biology

Background:

  • The retina is highly susceptible to mitochondrial dysfunction, leading to oxidative phosphorylation (OXPHOS) defects.
  • Retinal involvement manifests as retinal dystrophy (retinitis pigmentosa) or optic atrophy, often as part of complex syndromes.

Purpose of the Study:

  • To review the genetic causes of mitochondrial retinopathy, focusing on mutations in mitochondrial DNA (mtDNA) and nuclear genes.
  • To elucidate the clinical presentations and genetic underpinnings of mitochondrial vision disorders.

Main Methods:

  • Literature review of genetic mutations associated with mitochondrial retinopathy.
  • Analysis of clinical entities like retinitis pigmentosa, optic atrophy, NARP, MILS, KSS, and LHON.
  • Examination of both mtDNA and nuclear gene mutations, including OPA1.

Main Results:

  • mtDNA mutations are frequently linked to mitochondrial retinopathy, including NARP, MILS, KSS, and LHON.
  • Nuclear gene mutations also cause mitochondrial retinopathy, such as autosomal recessive LHON phenocopies and OPA1 mutations leading to optic atrophy.

Conclusions:

  • Mitochondrial retinopathy arises from diverse genetic defects, primarily in mtDNA but also in nuclear genes.
  • Understanding these genetic causes is crucial for diagnosing and potentially treating vision loss associated with mitochondrial disorders.