The DOI matches its Crossref record; 33 of the 33 references it cites were found in a registry or an index.
Muscle histone deacetylase 4 upregulation in amyotrophic lateral sclerosis: potential role in reinnervation ability and disease progression
Abstract
Amyotrophic lateral sclerosis is a typically rapidly progressive neurodegenerative disorder affecting motor neurons leading to progressive muscle paralysis and death, usually from respiratory failure, in 3-5 years. Some patients have slow disease progression and prolonged survival, but the underlying mechanisms remain poorly understood. Riluzole, the only approved treatment, only modestly prolongs survival and has no effect on muscle function. In the early phase of the disease, motor neuron loss is initially compensated for by collateral reinnervation, but over time this compensation fails, leading to progressive muscle wasting. The crucial role of muscle histone deacetylase 4 and its regulator microRNA-206 in compensatory reinnervation and disease progression was recently suggested in a mouse model of amyotrophic lateral sclerosis (transgenic mice carrying human mutations in the superoxide dismutase gene). Here, we sought to investigate whether the microRNA-206-histone deacetylase 4 pathway plays a role in muscle compensatory reinnervation in patients with amyotrophic lateral sclerosis and thus contributes to disease outcome differences. We studied muscle reinnervation using high-resolution confocal imaging of neuromuscular junctions in muscle samples obtained from 11 patients with amyotrophic lateral sclerosis, including five long-term survivors. We showed that the proportion of reinnervated neuromuscular junctions was significantly higher in long-term survivors than in pat…
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“miRNAs are increasingly recognized as key regulators in ALS pathophysiology, including mitochondrial integrity and neuroinflammation, with miR-155 and miR-206 demonstrating pivotal roles in SOD1G93A models [48,49,50,51].”
From Modulation of the miR-485-3p/PGC-1α Pathway by ASO-Loaded Nanoparticles Attenuates ALS Pathogenesis (2026) · cites this paper as [51]
Citations
Cites
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MicroRNA-206 Delays ALS Progression and Promotes Regeneration of Neuromuscular Synapses in Mice
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Research works printed but not linked: 1
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Cited by
Shukla, S., & Tekwani, B. L. (2020) · Frontiers in Pharmacology · doi:10.3389/fphar.2020.00537 · Cited by 314
Rossaert, E., Pollari, E., Jaspers, T., Van Helleputte, L., Jarpe, M., Van Damme, P., De Bock, K., Moisse, M., & Van Den Bosch, L. (2019) · Acta Neuropathologica Communications · doi:10.1186/s40478-019-0750-2 · Cited by 93
Calió, M. L., Henriques, E., Siena, A., Bertoncini, C. R. A., Gil-Mohapel, J., & Rosenstock, T. R. (2020) · Frontiers in Neuroscience · doi:10.3389/fnins.2020.00679 · Cited by 64
Burg, T., Rossaert, E., Moisse, M., Van Damme, P., & Van Den Bosch, L. (2021) · International Journal of Molecular Sciences · doi:10.3390/ijms222011224 · Cited by 50
Modulation of the miR-485-3p/PGC-1α Pathway by ASO-Loaded Nanoparticles Attenuates ALS Pathogenesis
Ryu, I. S., Ha, D.-I., Jung, Y.-J., Lee, H. J., Kim, I., Lim, Y. N., Min, H. S., Kim, S. H., Yoon, I., Cho, H.-J., et al. (2026) · International Journal of Molecular Sciences · doi:10.3390/ijms27020615 · Cited by 2
Metadata
- Authors
- Bruneteau, G., Simonet, T., Bauché, S., Mandjee, N., Malfatti, E., Girard, E., Tanguy, M.-L., Behin, A., Khiami, F., Sariali, E., Hell-Remy, C., Salachas, F., Pradat, P.-F., Fournier, E., Lacomblez, L., Koenig, J., Romero, N. B., Fontaine, B., Meininger, V., Schaeffer, L., & Hantaï, D.
- Venue
- Brain
- Publisher
- Oxford University Press (OUP)
- Published
- 2013-07-03
- Volume
- 136
- Issue
- 8
- Pages
- 2359-2368
- ISSN
- 1460-2156
- 0006-8950
- Cited by
- 131
- References
- 33
- Language
- en
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- The paper
- Bruneteau, G., Simonet, T., Bauché, S., Mandjee, N., Malfatti, E., Girard, E., Tanguy, M.-L., Behin, A., Khiami, F., Sariali, E., Hell-Remy, C., Salachas, F., Pradat, P.-F., Fournier, E., Lacomblez, L., Koenig, J., Romero, N. B., Fontaine, B., Meininger, V., . . . Hantaï, D. (2013). Muscle histone deacetylase 4 upregulation in amyotrophic lateral sclerosis: potential role in reinnervation ability and disease progression. Brain. https://doi.org/10.1093/brain/awt164
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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
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