Viral expression of ALS-linked ubiquilin-2 mutants causes inclusion pathology and behavioral deficits in mice
Open accessAbstract
BACKGROUND: UBQLN2 mutations have recently been associated with familial forms of amyotrophic lateral sclerosis (ALS) and ALS-dementia. UBQLN2 encodes for ubiquilin-2, a member of the ubiquitin-like protein family which facilitates delivery of ubiquitinated proteins to the proteasome for degradation. To study the potential role of ubiquilin-2 in ALS, we used recombinant adeno-associated viral (rAAV) vectors to express UBQLN2 and three of the identified ALS-linked mutants (P497H, P497S, and P506T) in primary neuroglial cultures and in developing neonatal mouse brains. RESULTS: In primary cultures rAAV2/8-mediated expression of UBQLN2 mutants resulted in inclusion bodies and insoluble aggregates. Intracerebroventricular injection of FVB mice at post-natal day 0 with rAAV2/8 expressing wild type or mutant UBQLN2 resulted in widespread, sustained expression of ubiquilin-2 in brain. In contrast to wild type, mutant UBQLN2 expression induced significant pathology with large neuronal, cytoplasmic inclusions and ubiquilin-2-positive aggregates in surrounding neuropil. Ubiquilin-2 inclusions co-localized with ubiquitin, p62/SQSTM, optineurin, and occasionally TDP-43, but were negative for α-synuclein, neurofilament, tau, and FUS. Mutant UBLQN2 expression also resulted in Thioflavin-S-positive inclusions/aggregates. Mice expressing mutant forms of UBQLN2 variably developed a motor phenotype at 3-4 months, including nonspecific clasping and rotarod deficits. CONCLUSIONS: These findings demonstrate that UBQLN2 mutants (P497H, P497S, and P506T) induce proteinopathy and cause behavioral deficits, supporting a "toxic" gain-of-function, which may contribute to ALS pathology. These data establish also that our rAAV model can be used to rapidly assess the pathological consequences of various UBQLN2 mutations and provides an agile system to further interrogate the molecular mechanisms of ubiquilins in neurodegeneration.
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Overexpression of ALS-linked UBQLN2 mutants triggers intracellular inclusions and motor defects in vitro and in vivo.
“while also triggering intracellular inclusions [226,227] and motor defects in vitro and in vivo [226].”
From Autophagy and ALS: mechanistic insights and therapeutic implications (2022) · cites this paper as [226]
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- Authors
- Ceballos-Diaz, C., Rosario, A. M., Park, H.-J., Chakrabarty, P., Sacino, A., Cruz, P. E., Siemienski, Z., Lara, N., Moran, C., Ravelo, N., Golde, T. E., & McFarland, N. R.
- Affiliations
- University of Florida (US)
- University of Florida Health (US)
- Corresponding author
- McFarland, Nikolaus R.
- Venue
- Molecular Neurodegeneration
- Published
- 2015-07-08
- Volume
- 10
- Issue
- 1
- Pages
- 25-25
- ISSN
- 1750-1326 (ISSN-L)
- PMC
- PMC4495639
- OpenAlex
- W1750731840
- Cited by
- 58
- References
- 26
- Citations by year
- 2026: 2 · 2025: 2 · 2024: 3 · 2023: 5 · 2022: 4 · 2021: 10 · 2020: 5 · 2019: 7 · 2018: 8 · 2017: 5 · 2016: 6 · 2015: 1
- FWCI
- 4.70
- Type
- journal article
- Language
- en
- Open access
- gold
- doi.org
- Locations
- Molecular Neurodegeneration (publishedVersion, cc-by)
- PubMed Central (publishedVersion, cc-by)
- PubMed (publishedVersion)
- Funders
- Ellison Medical Foundation
- National Institutes of Health
- National Institute of Neurological Disorders and Stroke (K08NS067024, NS067024)
- Topics
- Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Genetic Neurodegenerative Diseases
- Health Sciences › Medicine › Physiology › Alzheimer's disease research and treatments
- Keywords
- UBQLN2, amyotrophic lateral sclerosis, familial ALS, protein aggregation, gain-of-function toxicity, intracerebroventricular administration, motor impairment, ubiquitin-like proteins, optineurin, TDP-43
- MeSH terms
- Autophagy-Related Proteins, Dependovirus, Amyotrophic Lateral Sclerosis, Animals, Animals, Newborn, Brain, Inclusion Bodies, Disease Models, Animal, Genes, Synthetic, Genetic Vectors, Humans, Injections, Intraventricular, Mice, Transgenic, Nerve Tissue Proteins, Neurons, Transduction, Genetic, Ubiquitins, Point Mutation, Hand Strength, Cell Cycle Proteins, Transgenes, Mutation, Missense, Rotarod Performance Test, Proteasome Endopeptidase Complex, Adaptor Proteins, Signal Transducing, Mice, Ubiquitination, HEK293 Cells, Proteolysis
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
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