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Reduced C9ORF72 function exacerbates gain of toxicity from ALS/FTD-causing repeat expansion in C9orf72
Open accessAbstract
Hexanucleotide expansions in C9orf72, which encodes a predicted guanine exchange factor, are the most frequent genetic cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Although repeat expansion has been established to generate toxic products, mRNAs encoding the C9ORF72 protein are also reduced in affected individuals. In this study, we tested how C9ORF72 protein levels affected repeat-mediated toxicity. In somatic transgenic mice expressing 66 GGGGCC repeats, inactivation of one or both endogenous C9orf72 alleles provoked or accelerated, respectively, early death. In mice expressing a C9orf72 transgene with 450 repeats that did not encode the C9ORF72 protein, inactivation of one or both endogenous C9orf72 alleles exacerbated cognitive deficits, hippocampal neuron loss, glial activation and accumulation of dipeptide-repeat proteins from translation of repeat-containing RNAs. Reduced C9ORF72 was shown to suppress repeat-mediated elevation in autophagy. These efforts support a disease mechanism in ALS/FTD resulting from reduced C9ORF72, which can lead to autophagy deficits, synergizing with repeat-dependent gain of toxicity.
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“Furthermore, the loss of C9orf72 function and consequent impairment in autophagy may enhance DPR accumulation in C9orf72 mutation carriers, acting synergistically to accentuate neurodegeneration [154,172,173].”
From Autophagy and ALS: mechanistic insights and therapeutic implications (2022) · cites this paper as [173]
In the citing paper
“On the other hand, loss of endogenous murine C9orf72 results in glial activation, impaired autophagy and toxicity when combined with the C9orf72 expansion mutation [173].”
From Autophagy and ALS: mechanistic insights and therapeutic implications (2022) · cites this paper as [173]
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“Consistently, it has been recently observed that loss of wild‐type C9orf72 function exacerbates the neurotoxic effects of a C9orf72 mutant allele, bearing hexanucleotide expansions, by repressing autophagy (Zhu et al, 2020).”
From Autophagy in major human diseases (2021) · cites this paper as Zhu et al, 2020
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Metadata
- Authors
- Zhu, Q., Jiang, J., Gendron, T. F., McAlonis-Downes, M., Jiang, L., Taylor, A., Diaz Garcia, S., Ghosh Dastidar, S., Rodriguez, M. J., King, P., Zhang, Y., La Spada, A. R., Xu, H., Petrucelli, L., Ravits, J., Da Cruz, S., Lagier-Tourenne, C., & Cleveland, D. W.
- Affiliations
- University of California San Diego (US)
- Emory University (US)
- Mayo Clinic in Florida (US)
- Sanford Burnham Prebys Medical Discovery Institute (US)
- Corresponding author
- Cleveland, Don W.
- ORCID
- Venue
- Nature Neuroscience
- Published
- 2020-04-13
- Volume
- 23
- Issue
- 5
- Pages
- 615-624
- ISSN
- 1097-6256 (ISSN-L)
- 1546-1726
- PMC
- PMC7384305
- OpenAlex
- W3015315508
- Cited by
- 220
- References
- 60
- Citations by year
- 2026: 10 · 2025: 26 · 2024: 28 · 2023: 40 · 2022: 35 · 2021: 49 · 2020: 31
- FWCI
- 16.88
- Type
- journal article
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Locations
- Nature Neuroscience (publishedVersion)
- PubMed (publishedVersion)
- PubMed Central (acceptedVersion)
- eScholarship (California Digital Library) (submittedVersion)
- Lirias (KU Leuven) (submittedVersion)
- Funders
- Amyotrophic Lateral Sclerosis Association (18-IIA-407, 2313)
- Muscular Dystrophy Association (479769)
- U.S. Department of Health & Human Services | NIH | National Institute of Neurological Disorders and Stroke (P01-NS099114, P01-NS084974, R01-NS088689, R01-NS27036, R01-NS087227)
- Target ALS
- Topics
- Health Sciences › Medicine › Clinical Neurology › Amyotrophic Lateral Sclerosis Research
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Genetic Neurodegenerative Diseases
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Hereditary Neurological Disorders
- Keywords
- C9orf72, hexanucleotide repeat expansion, amyotrophic lateral sclerosis, frontotemporal dementia, dipeptide repeat proteins, autophagy, transgenic mouse model, cognitive impairment, hippocampal neuronal loss, glial activation
- MeSH terms
- C9orf72 Protein, Amyotrophic Lateral Sclerosis, Animals, Disease Models, Animal, Female, Male, Mice, Inbred C57BL, Mice, Transgenic, DNA Repeat Expansion, Mice, Frontotemporal Dementia
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