ArticleAnnals of neurology2020
Absence of Subcerebral Projection Neurons Is Beneficial in a Mouse Model of Amyotrophic Lateral Sclerosis.
Article in Annals of neurology, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers.
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Who cites it
16 citing papers in PubMed, 23 citations in OpenAlex.
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- Development and validation a novel FEZF2 based fluorescent reporter for corticospinal motor neurons.Metabolic brain disease · 2024Article
- Clinical spreading of muscle weakness in amyotrophic lateral sclerosis (ALS): a study in 910 patients.Journal of neurology · 2024Observational
- Rare predicted deleterious FEZF2 variants are associated with a neurodevelopmental phenotype.American journal of medical genetics. Part A · 2024Article
- Novel rAAV vector mediated intrathecal HGF delivery has an impact on neuroimmune modulation in the ALS motor cortex with TDP-43 pathology.Gene therapy · 2023Article
- The evidence against somatotopic organization of function in the primate corticospinal tract.Brain : a journal of neurology · 2023Review
- Cortical glia in SOD1(G93A) mice are subtly affected by ALS-like pathology.Scientific reports · 2023Article
- Upper motor neurons are a target for gene therapy and UCHL1 is necessary and sufficient to improve cellular integrity of diseased upper motor neurons.Gene therapy · 2022Article
- NU-9 improves health of hSOD1Scientific reports · 2022Article
- Advances in Gene Delivery Methods to Label and Modulate Activity of Upper Motor Neurons: Implications for Amyotrophic Lateral Sclerosis.Brain sciences · 2021Review
- Differential Epigenetic Signature of Corticospinal Motor Neurons in ALS.Brain sciences · 2021Article
- Neurophysiological Mechanisms Underlying Cortical Hyper-Excitability in Amyotrophic Lateral Sclerosis: A Review.Brain sciences · 2021Review
- Upper and Lower Motor Neuron Degenerations Are Somatotopically Related and Temporally Ordered in theBrain sciences · 2021Article
- Improving mitochondria and ER stability helps eliminate upper motor neuron degeneration that occurs due to mSOD1 toxicity and TDP-43 pathology.Clinical and translational medicine · 2021Article
- Cortical Circuit Dysfunction as a Potential Driver of Amyotrophic Lateral Sclerosis.Frontiers in neuroscience · 2020Review
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Authors and funding
9 authors at 3 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
objectiveRecent studies carried out on amyotrophic lateral sclerosis patients suggest that the disease might initiate in the motor cortex and spread to its targets along the corticofugal tracts. In this study, we aimed to test the corticofugal hypothesis of amyotrophic lateral sclerosis experimentally.
methodsSod1
resultsAbsence of subcerebral projection neurons delayed disease onset, reduced weight loss and motor impairment, and increased survival without modifying disease duration. Absence of corticospinal neurons also limited presymptomatic hyper-reflexia, a typical component of the upper motoneuron syndrome.
interpretationMajor corticofugal tracts are crucial to the onset and progression of amyotrophic lateral sclerosis. In the context of the disease, subcerebral projection neurons might carry detrimental signals to their downstream targets. In its entirety, this study provides the first experimental arguments in favor of the corticofugal hypothesis of amyotrophic lateral sclerosis. ANN NEUROL 2020;88:688-702.
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