The DOI matches its Crossref record; 11 of the 11 references it cites were found in a registry or an index.
Loss of ALS-associated TDP-43 in zebrafish causes muscle degeneration, vascular dysfunction, and reduced motor neuron axon outgrowth
Open accessAbstract
Mutations in the Tar DNA binding protein of 43 kDa (TDP-43; TARDBP) are associated with amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration with TDP-43+inclusions (FTLD-TDP). To determine the physiological function of TDP-43, we knocked out zebrafish Tardbp and its paralogue Tardbp (TAR DNA binding protein-like), which lacks the glycine-rich domain where ALS- and FTLD-TDP–associated mutations cluster.tardbp mutants show no phenotype, a result of compensation by a unique splice variant of tardbpl that additionally contains a C-terminal elongation highly homologous to the glycine-rich domain of tardbp. Double-homozygous mutants of tardbp and tardbpl show muscle degeneration, strongly reduced blood circulation, mispatterning of vessels, impaired spinal motor neuron axon outgrowth, and early death. In double mutants the muscle-specific actin binding protein Filamin Ca is up-regulated. Strikingly,Filamin C is similarly increased in the frontal cortex of FTLD-TDP patients, suggesting aberrant expression in smooth muscle cells and TDP-43 loss-of-function as one underlying disease mechanism.
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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
Kiernan, M. C., Vucic, S., Cheah, B. C., Turner, M. R., Eisen, A., Hardiman, O., Burrell, J. R., & Zoing, M. C. (2011) · The Lancet · doi:10.1016/s0140-6736(10)61156-7 · Cited by 2,603
Bell, R. D., Winkler, E. A., Sagare, A. P., Singh, I., LaRue, B., Deane, R., & Zlokovic, B. V. (2010) · Neuron · doi:10.1016/j.neuron.2010.09.043 · Cited by 1,448
Long pre-mRNA depletion and RNA missplicing contribute to neuronal vulnerability from loss of TDP-43
Polymenidou, M., Lagier-Tourenne, C., Hutt, K. R., Huelga, S. C., Moran, J., Liang, T. Y., Ling, S.-C., Sun, E., Wancewicz, E., Mazur, C., et al. (2011) · Nature Neuroscience · doi:10.1038/nn.2779 · Cited by 1,335
Common mechanisms of nerve and blood vessel wiring
Carmeliet, P., & Tessier-Lavigne, M. (2005) · Nature · doi:10.1038/nature03875 · Cited by 1,085
ALS-causing SOD1 mutants generate vascular changes prior to motor neuron degeneration
Zhong, Z., Deane, R., Ali, Z., Parisi, M., Shapovalov, Y., O'Banion, M. K., Stojanovic, K., Sagare, A., Boillee, S., Cleveland, D. W., et al. (2008) · Nature Neuroscience · doi:10.1038/nn2073 · Cited by 476
TDP-43 Is a Developmentally Regulated Protein Essential for Early Embryonic Development
Sephton, C. F., Good, S. K., Atkin, S., Dewey, C. M., Mayer, P., Herz, J., & Yu, G. (2010) · Journal of Biological Chemistry · doi:10.1074/jbc.m109.061846 · Cited by 370
Blood–spinal cord barrier breakdown and pericyte reductions in amyotrophic lateral sclerosis
Winkler, E. A., Sengillo, J. D., Sullivan, J. S., Henkel, J. S., Appel, S. H., & Zlokovic, B. V. (2013) · Acta Neuropathologica · doi:10.1007/s00401-012-1039-8 · Cited by 354
Loss of murine TDP-43 disrupts motor function and plays an essential role in embryogenesis
Kraemer, B. C., Schuck, T., Wheeler, J. M., Robinson, L. C., Trojanowski, J. Q., Lee, V. M. Y., & Schellenberg, G. D. (2010) · Acta Neuropathologica · doi:10.1007/s00401-010-0659-0 · Cited by 350
Cited by
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
Efficient CRISPR/Cas9 genome editing with low off-target effects in zebrafish
Hruscha, A., Krawitz, P., Rechenberg, A., Heinrich, V., Hecht, J., Haass, C., & Schmid, B. (2013) · Development · doi:10.1242/dev.099085 · Cited by 478
Self-citationScotter, E. L., Vance, C., Nishimura, A. L., Lee, Y.-B., Chen, H.-J., Urwin, H., Sardone, V., Mitchell, J. C., Rogelj, B., Rubinsztein, D. C., et al. (2014) · Journal of Cell Science · doi:10.1242/jcs.140087 · Cited by 268
Triad of TDP43 control in neurodegeneration: autoregulation, localization and aggregation
Tziortzouda, P., Van Den Bosch, L., & Hirth, F. (2021) · Nature Reviews Neuroscience · doi:10.1038/s41583-021-00431-1 · Cited by 267
Eichner, R., Heider, M., Fernández-Sáiz, V., van Bebber, F., Garz, A.-K., Lemeer, S., Rudelius, M., Targosz, B.-S., Jacobs, L., Knorn, A.-M., et al. (2016) · Nature Medicine · doi:10.1038/nm.4128 · Cited by 183
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Metadata
- Authors
- Schmid, B., Hruscha, A., Hogl, S., Banzhaf-Strathmann, J., Strecker, K., van der Zee, J., Teucke, M., Eimer, S., Hegermann, J., Kittelmann, M., Kremmer, E., Cruts, M., Solchenberger, B., Hasenkamp, L., van Bebber, F., Van Broeckhoven, C., Edbauer, D., Lichtenthaler, S. F., & Haass, C.
- Venue
- Proceedings of the National Academy of Sciences
- Published
- 2013-03
- Volume
- 110
- Issue
- 13
- Pages
- 4986-4991
- ISSN
- 0027-8424 (ISSN-L)
- 1091-6490
- OpenAlex
- W2107338709
- Cited by
- 169
- References
- 11
- FWCI
- 7.60
- Type
- journal article
- Language
- en
- Open access
- green
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- Schmid, B., Hruscha, A., Hogl, S., Banzhaf-Strathmann, J., Strecker, K., van der Zee, J., Teucke, M., Eimer, S., Hegermann, J., Kittelmann, M., Kremmer, E., Cruts, M., Solchenberger, B., Hasenkamp, L., van Bebber, F., Van Broeckhoven, C., Edbauer, D., Lichtenthaler, S. F., & Haass, C. (2013). Loss of ALS-associated TDP-43 in zebrafish causes muscle degeneration, vascular dysfunction, and reduced motor neuron axon outgrowth. Proceedings of the National Academy of Sciences. https://doi.org/10.1073/pnas.1218311110
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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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