Cited by 189Amyotrophic Lateral Sclerosis ResearchAutophagy in Disease and TherapyHereditary Neurological Disorders

Distinct roles for motor neuron autophagy early and late in the SOD1 G93A mouse model of ALS

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Rudnick, Noam D., Griffey, Christopher J., Guarnieri, Paolo, Gerbino, Valeria, Wang, Xueyong, Piersaint, Jason A., Tapia, Juan Carlos, Rich, Mark M. +1 more·2017·Proceedings of the National Academy of Sciences

Abstract

Significance: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that leads to death of spinal motor neurons and paralysis. Genetic studies of ALS patients have identified mutations in autophagy pathway genes including p62 / SQSTM1 , OPTN , TBK1 , VCP , and C9ORF72 . However, the mechanisms by which these mutations cause ALS are not understood. Here we investigated the role of autophagy in a mouse model of ALS by specifically disrupting the critical autophagy gene Atg7 in motor neurons. We found that inhibition of autophagy in motor neurons accelerated disease onset but prolonged lifespan. This increase in longevity was associated with a reduction in glial inflammation and interneuron pathology, indicating a non–cell-autonomous role for motor neuron autophagy in disease pathogenesis.

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Crossref · 10.1073/pnas.1704294114

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  • Impaired autophagy sensitizes the nervous system to key pathogenic events occurring early in ALS pathogenesis.

    “These findings not only suggest that impaired autophagy sensitizes the nervous system to key pathogenic events occurring early in ALS pathogenesis”
  • Conditional deletion of Atg7 in mouse motor neurons accentuates early ALS pathology but mitigates glial inflammation and early death.

    “conditional deletion of Atg7 in mouse motor neurons accentuates early ALS pathology but mitigates glial inflammation and early death [336].”

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Authors
Rudnick, N. D., Griffey, C. J., Guarnieri, P., Gerbino, V., Wang, X., Piersaint, J. A., Tapia, J. C., Rich, M. M., & Maniatis, T.
Affiliations
  • Columbia University Irving Medical Center (US)
  • Wright State University (US)
Venue
Proceedings of the National Academy of Sciences
Published
2017-09-13
Volume
114
Issue
39
Pages
E8294-E8303
ISSN
  • 0027-8424 (ISSN-L)
  • 1091-6490
OpenAlex
W2755399567
Cited by
189
References
64
Citations by year
2026: 11 · 2025: 16 · 2024: 14 · 2023: 17 · 2022: 18 · 2021: 35 · 2020: 26 · 2019: 27 · 2018: 21 · 2017: 4
FWCI
14.85
Type
journal article
Language
en
Open access
Locations
  • Proceedings of the National Academy of Sciences (publishedVersion)
  • PubMed Central (publishedVersion)
  • PubMed (publishedVersion)
  • CORE Scholar (Wright State University) (submittedVersion)
Funders
  • HHS | NIH | NIH Office of the Director (DP1 OD003930)
  • HHS | NIH | National Institute of Neurological Disorders and Stroke (F31 NS089131, R01 NS082354)
  • HHS | NIH | National Institute of General Medical Sciences (T32 GM007367)
Topics
  • Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
  • Health Sciences › Medicine › Epidemiology › Autophagy in Disease and Therapy
  • Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Hereditary Neurological Disorders
Keywords
amyotrophic lateral sclerosis, ATG7, SOD1 G93A mouse model, autophagy inhibition, glial inflammation, lifespan extension, motor neuron degeneration
MeSH terms
Autophagy-Related Protein 7, Superoxide Dismutase-1, Amyotrophic Lateral Sclerosis, Animals, Autophagy, Disease Models, Animal, Motor Neurons, Superoxide Dismutase, Mice, Knockout, Mice

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