ArticleMolecular therapy : the journal of the American Society of Gene Therapy2014
Neuroglobin gene therapy prevents optic atrophy and preserves durably visual function in Harlequin mice.
Article in Molecular therapy : the journal of the American Society of Gene Therapy, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers.
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Who cites it
15 citing papers in PubMed, 31 citations in OpenAlex.
- Targeting Retinal Neuroglial Vascular Unit Damage: Novel Therapeutic Strategies for Early-Stage Diabetic Retinopathy.Journal of diabetes research · 2025Review
- Neuroglobin overexpression in cerebellar neurons of Harlequin mice improves mitochondrial homeostasis and reduces ataxic behavior.Molecular therapy : the journal of the American Society of Gene Therapy · 2024Article
- Article
- A rare natural lipid induces neuroglobin expression to prevent amyloid oligomers toxicity and retinal neurodegeneration.Aging cell · 2022Article
- Structural and (Pseudo-)Enzymatic Properties of Neuroglobin: Its Possible Role in Neuroprotection.Cells · 2021Review
- Review
- Exercise Training and Neurodegeneration in Mitochondrial Disorders: Insights From the Harlequin Mouse.Frontiers in physiology · 2020Article
- Neuroglobin Expression in the Brain: a Story of Tissue Homeostasis Preservation.Molecular neurobiology · 2019Review
- Physical Exercise and Mitochondrial Disease: Insights From a Mouse Model.Frontiers in neurology · 2019Article
- Manual Acupuncture for Optic Atrophy: A Systematic Review and Meta-Analysis.Evidence-based complementary and alternative medicine : eCAM · 2019Review
- Neuroglobin Expression Models as a Tool to Study Its Function.Oxidative medicine and cellular longevity · 2019Review
- Neuroglobin Can Prevent or Reverse Glaucomatous Progression in DBA/2J Mice.Molecular therapy. Methods & clinical development · 2017Article
- Bilateral neuroinflammatory processes in visual pathways induced by unilateral ocular hypertension in the rat.Journal of neuroinflammation · 2016Article
- Nuclear expression of mitochondrial ND4 leads to the protein assembling in complex I and prevents optic atrophy and visual loss.Molecular therapy. Methods & clinical development · 2015Article
- Vector platforms for gene therapy of inherited retinopathies.Progress in retinal and eye research · 2014Review
Corrections and comments
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Authors and funding
10 authors at 7 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Neuroglobin (NGB) is considered as an endogenous neuroprotective molecule against stroke, since the protein alleviates the adverse effects of hypoxic and ischemic insults. We previously demonstrated the functional link between NGB and mitochondria since it is required for respiratory chain function. Thus, here, we evaluated the relevance of this effect in the Harlequin (Hq) mouse strain, which exhibits retinal ganglion cell (RGC) loss and optic atrophy due to a respiratory chain complex I (CI) defect. A twofold decrease of NGB amounts was observed in Hq retinas. We constructed a recombinant adeno-associated virus which combines to the mouse NGB open reading frame, its 5' and 3'UTR, for guarantying mRNA stability and translation capacity. The vector was administrated intravitreally to Hq mice and NGB expression was stable for up to 7 months without negative effect on retinal architecture or function. On the contrary, RGCs and their axons were substantially preserved from degeneration; consequently, CI activity in optic nerves was protected conferring improvements in vision. Hence, we established that NGB prevents respiratory chain impairment, therefore, protecting visual function otherwise compromised by mitochondrial energetic failure.
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