ArticleScientific reports2016
Neuoroprotective efficacies by KUS121, a VCP modulator, on animal models of retinal degeneration.
Article in Scientific reports, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers.
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Who cites it
22 citing papers in PubMed, 31 citations in OpenAlex.
- Post hoc analysis of a randomized placebo-controlled trial suggests potential visual benefits of branched-chain amino acids in retinitis pigmentosa.Scientific reports · 2025Trial
- Efficacy and Safety of Branched Chain Amino Acids on Retinitis Pigmentosa: A Randomized, Double-Blind, Placebo-Controlled Clinical Trial.Translational vision science & technology · 2024Trial
- Trial
- Safety and neuroprotective efficacy of the VCP inhibitor ML240 in large-animal and human retinal explants: a preclinical ex vivo study.BMC medicine · 2026Article
- Valosin-containing protein modulator KUS121 protects retinal neurons in a rat model of anterior ischemic optic neuropathy.Scientific reports · 2025Article
- The effect of KUS121, a novel VCP modulator, against ischemic injury in random pattern flaps.PloS one · 2024Article
- Involvement of endothelins in neuroprotection of valosin-containing protein modulators against retinal ganglion cell damage.Scientific reports · 2022Article
- Branched Chain Amino Acids Promote ATP Production Via Translocation of Glucose Transporters.Investigative ophthalmology & visual science · 2022Article
- NAMPT-dependent NADCell death discovery · 2022Article
- Mitochondrial quality control in the brain: The physiological and pathological roles.Frontiers in neuroscience · 2022Review
- KUS121 attenuates the progression of monosodium iodoacetate-induced osteoarthritis in rats.Scientific reports · 2021Article
- A VCP modulator, KUS121, as a promising therapeutic agent for post-traumatic osteoarthritis.Scientific reports · 2020Article
- Effect of VCP modulators on gene expression profiles of retinal ganglion cells in an acute injury mouse model.Scientific reports · 2020Article
- KUS121, a valosin-containing protein modulator, attenuates ischemic stroke via preventing ATP depletion.Scientific reports · 2019Article
- Adenosine triphosphate maintenance by branched chain amino acids as a novel neuroprotective strategy for retinal neurodegenerative diseases.Neural regeneration research · 2019Article
- Assessment of Central Visual Function in Patients with Retinitis Pigmentosa.Scientific reports · 2018Article
- KUS121, an ATP regulator, mitigates chorioretinal pathologies in animal models of age-related macular degeneration.Heliyon · 2018Article
- Branched chain amino acids attenuate major pathologies in mouse models of retinal degeneration and glaucoma.Heliyon · 2018Article
- Modulation of valosin-containing protein by Kyoto University Substances (KUS) as a novel therapeutic strategy for ischemic neuronal diseases.Neural regeneration research · 2017Review
- ATP Maintenance via Two Types of ATP Regulators Mitigates Pathological Phenotypes in Mouse Models of Parkinson's Disease.EBioMedicine · 2017Article
Corrections and comments
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Authors and funding
8 authors at 4 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Retinitis pigmentosa (RP) is one of the leading causes of adult blindness and has no established therapy. We have shown that valosin-containing protein (VCP) modulators, Kyoto University Substances (KUSs), ameliorated abnormally low ATP levels by inhibiting the ATPase of VCP, thereby protected several types of cells, including retinal neurons, from cell death-inducing insults. In this study, we found that KUS121, one of the VCP modulators, effectively protects photoreceptors both morphologically and functionally, in two animal models of retinal degeneration, rd12 mice and RP rabbits with a rhodopsin (Pro347Leu) mutation. In rd12 mice, KUS121 suppressed the loss of photoreceptors, not only rods but also cones, as well as the visual function deterioration. Significant protective effects existed even when the medication was started in later stages of the disease. In RP rabbits, KUS121 suppressed thinning of the outer nuclear layer and maintained visual function. In the retinas treated with KUS121, suppression of endoplasmic reticulum stress, activation of mammalian target of rapamycin and suppression of disease-associated apoptosis were evident. The ability of KUS121 to protect photoreceptors, especially cones, even in later stages of the disease may contribute to the preservation of central vision in RP patients, which is important for quality of vision.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.