The DOI matches its Crossref record; 4 of the 4 references it cites were found in a registry or an index.
Depletion of TDP‐43 affects Drosophila motoneurons terminal synapsis and locomotive behavior
Abstract
Pathological modifications in the highly conserved and ubiquitously expressed heterogeneous ribonucleoprotein TDP‐43 were recently associated to neurodegenerative diseases including amyotrophic lateral sclerosis (ALS), a late‐onset disorder that affects predominantly motoneurons [Neumann, M. et al. (2006) Ubiquitinated TDP‐43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science 314, 130–133, Sreedharan, J. et al. (2008) TDP‐43 mutations in familial and sporadic amyotrophic lateral sclerosis. Science 319, 1668–1672, Kabashi, E. et al. (2008) TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis. Nat. Genet. 40, 572–574]. However, the function of TDP‐43 in vivo is unknown and a possible direct role in neurodegeneration remains speculative. Here, we report that flies lacking Drosophila TDP‐43 appeared externally normal but presented deficient locomotive behaviors, reduced life span and anatomical defects at the neuromuscular junctions. These phenotypes were rescued by expression of the human protein in a restricted group of neurons including motoneurons. Our results demonstrate the role of this protein in vivo and suggest an alternative explanation to ALS pathogenesis that may be more due to the lack of TDP 43 function than to the toxicity of the aggregates.
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Cites
Ubiquitinated TDP-43 in Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis
Neumann, M., Sampathu, D. M., Kwong, L. K., Truax, A. C., Micsenyi, M. C., Chou, T. T., Bruce, J., Schuck, T., Grossman, M., Clark, C. M., et al. (2006) · Science · doi:10.1126/science.1134108 · Cited by 6,576
Arai, T., Hasegawa, M., Akiyama, H., Ikeda, K., Nonaka, T., Mori, H., Mann, D., Tsuchiya, K., Yoshida, M., Hashizume, Y., et al. (2006) · Biochemical and Biophysical Research Communications · doi:10.1016/j.bbrc.2006.10.093 · Cited by 2,732
TDP-43 Mutations in Familial and Sporadic Amyotrophic Lateral Sclerosis
Sreedharan, J., Blair, I. P., Tripathi, V. B., Hu, X., Vance, C., Rogelj, B., Ackerley, S., Durnall, J. C., Williams, K. L., Buratti, E., et al. (2008) · Science · doi:10.1126/science.1154584 · Cited by 2,630
Self-citationClinical and Pathological Continuum of Multisystem TDP-43 Proteinopathies
Geser, F., Martinez-Lage, M., Robinson, J., Uryu, K., Neumann, M., Brandmeir, N. J., Xie, S. X., Kwong, L. K., Elman, L., McCluskey, L., et al. (2009) · Archives of Neurology · doi:10.1001/archneurol.2008.558 · Cited by 267
Cited by
TDP-43 and FUS/TLS: emerging roles in RNA processing and neurodegeneration
Lagier-Tourenne, C., Polymenidou, M., & Cleveland, D. W. (2010) · Human Molecular Genetics · doi:10.1093/hmg/ddq137 · Cited by 1,021
The changing scene of amyotrophic lateral sclerosis
Robberecht, W., & Philips, T. (2013) · Nature Reviews Neuroscience · doi:10.1038/nrn3430 · Cited by 957
TDP-43 repression of nonconserved cryptic exons is compromised in ALS-FTD
Ling, J. P., Pletnikova, O., Troncoso, J. C., & Wong, P. C. (2015) · Science · doi:10.1126/science.aab0983 · Cited by 688
Axonal Transport of TDP-43 mRNA Granules Is Impaired by ALS-Causing Mutations
Alami, N. H., Smith, R. B., Carrasco, M. A., Williams, L. A., Winborn, C. S., Han, S. S. W., Kiskinis, E., Winborn, B., Freibaum, B. D., Kanagaraj, A., et al. (2014) · Neuron · doi:10.1016/j.neuron.2013.12.018 · Cited by 660
Gains or losses: molecular mechanisms of TDP43-mediated neurodegeneration
Lee, E. B., Lee, V. M.-Y., & Trojanowski, J. Q. (2012) · Nature Reviews Neuroscience · doi:10.1038/nrn3121 · Cited by 659
Metadata
- Authors
- Feiguin, F., Godena, V. K., Romano, G., D'Ambrogio, A., Klima, R., & Baralle, F. E.
- Venue
- FEBS Letters
- Publisher
- Wiley
- Published
- 2009-04-18
- Volume
- 583
- Issue
- 10
- Pages
- 1586-1592
- ISSN
- 0014-5793 (ISSN-L)
- 1873-3468
- OpenAlex
- W2091145925
- Cited by
- 306
- References
- 4
- FWCI
- 16.50
- Type
- journal article
- Language
- en
- Open access
- closed
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- Feiguin, F., Godena, V. K., Romano, G., D'Ambrogio, A., Klima, R., & Baralle, F. E. (2009). Depletion of TDP‐43 affects Drosophila motoneurons terminal synapsis and locomotive behavior. FEBS Letters. https://doi.org/10.1016/j.febslet.2009.04.019
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Bibliographic data: Semantic Scholar, OpenAlex, Crossref.
Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.
