Nitric Oxide Signaling Is Disrupted in the Yeast Model for Batten Disease
Open accessAbstract
The juvenile form of neuronal ceroid lipofuscinoses (JNCLs), or Batten disease, results from mutations in the CLN3 gene, and it is characterized by the accumulation of lipopigments in the lysosomes of several cell types and by extensive neuronal death. We report that the yeast model for JNCL (btn1-Δ) that lacks BTN1, the homologue to human CLN3, has increased resistance to menadione-generated oxidative stress. Expression of human CLN3 complemented the btn1-Δ phenotype, and equivalent Btn1p/Cln3 mutations correlated with JNCL severity. We show that the previously reported decreased levels of l-arginine in btn1-Δ limit the synthesis of nitric oxide (·NO) in both physiological and oxidative stress conditions. This defect in ·NO synthesis seems to suppress the signaling required for yeast menadione-induced apoptosis, thus explaining btn1-Δ phenotype of increased resistance. We propose that in JNCL, a limited capacity to synthesize ·NO directly caused by the absence of Cln3 function may contribute to the pathology of the disease.
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There have been a number of publications reporting the existence of NOS-like effect in bacteria, yeast, and plants.
“Nevertheless, there have been a number of publications reporting the existence of NOS-like effect in bacteria [15,16], yeast [17,18], and plants [19].”
From Effect of Nitric Oxide on the Antifungal Activity of Oxidative Stress and Azoles Against Candida albicans (2016) · cites this paper as [17]
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References 63 · Cited by 57
- Authors
- Osório, N. S., Carvalho, A., Almeida, A. J., Padilla-Lopez, S., Leão, C., Laranjinha, J., Ludovico, P., Pearce, D. A., & Rodrigues, F.
- Affiliations
- University of Minho (PT)
- Center for Aging and Developmental Biology, Aab Institute of Biomedical Sciences, and Departments of Biochemistry and Biophysics and Neurology, University of Rochester School of Medicine and Dentistry, Rochester, NY 14642; and
- *Life and Health Sciences Research Institute (ICVS), School of Health Sciences, University of Minho, 4710 Braga, Portugal;
- Centro de Neurociências e Biologia Celular (PT)
- University of Rochester Medicine (US)
- Corresponding author
- Rodrigues, Fernando
- ORCID
- Venue
- Molecular Biology of the Cell
- Published
- 2007-07
- Volume
- 18
- Issue
- 7
- Pages
- 2755-2767
- ISSN
- 1059-1524 (ISSN-L)
- 1939-4586
- PMC
- PMC1924819
- OpenAlex
- W2156040170
- Cited by
- 57
- References
- 63
- Citations by year
- 2024: 1 · 2023: 1 · 2022: 1 · 2021: 1 · 2020: 2 · 2019: 2 · 2018: 2 · 2017: 2 · 2016: 4 · 2015: 3 · 2014: 2 · 2013: 8 · 2012: 5
- FWCI
- 4.01
- Type
- journal article
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Locations
- Molecular Biology of the Cell (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- Portuguese National Funding Agency for Science, Research and Technology (RCAAP Project by FCT) (submittedVersion, other-oa)
- Portuguese National Funding Agency for Science, Research and Technology (RCAAP Project by FCT) (submittedVersion, cc-by-nc-nd)
- Funders
- National Institutes of Health (R01 NS)
- Fundação para a Ciência e a Tecnologia (POCI/BIA-BCM/57364/2004)
- National Institute of Neurological Disorders and Stroke (R01NS036610, R01 NS36610)
- Topics
- Health Sciences › Medicine › Physiology › Lysosomal Storage Disorders Research
- Health Sciences › Medicine › Rheumatology › Glycogen Storage Diseases and Myoclonus
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
- Keywords
- Batten disease, CLN3, nitric oxide signaling, L-arginine, oxidative stress, menadione, apoptosis, yeast model, neuronal cell death, gene complementation
- MeSH terms
- Cell Nucleus, Humans, Models, Biological, Mutation, Neuronal Ceroid-Lipofuscinoses, Nitric Oxide, Saccharomyces cerevisiae, Time Factors, Signal Transduction, Biomarkers, Cyclins, Apoptosis, Reactive Oxygen Species, Oxidative Stress, Vitamin K 3, Saccharomyces cerevisiae Proteins, Microbial Viability
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