The DOI matches its Crossref record; 16 of the 16 references it cites were found in a registry or an index.
Progranulin Mediates Caspase-Dependent Cleavage of TAR DNA Binding Protein-43
Open accessAbstract
TAR DNA binding protein-43 (TDP-43) is the pathologic substrate of neuronal and glial inclusions in frontotemporal lobar degeneration with ubiquitin-positive inclusions (FTDL-U) and in amyotrophic lateral sclerosis (ALS). Mutations in the progranulin gene (PGRN) have been shown to cause familial FTLD-U. The relationship between progranulin and TDP-43 and their respective roles in neurodegeneration is unknown. We report that progranulin mediates proteolytic cleavage of TDP-43 to generate ∼35 and ∼25 kDa species. Suppression of PGRN expression with small interfering RNA leads to caspase-dependent accumulation of TDP-43 fragments that can be inhibited with caspase inhibitor treatment. Cells treated with staurosporine also induced caspase-dependent cleavage and redistribution of TDP-43 from its nuclear localization to cytoplasm. Altered cleavage and redistribution of TDP-43 in cell culture models are similar to findings in FTLD-U and ALS. The results suggest that abnormal metabolism of TDP-43 mediated by progranulin may play a pivotal role in neurodegeneration.
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Mutations in progranulin cause tau-negative frontotemporal dementia linked to chromosome 17
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Self-citationConversion of Proepithelin to Epithelins
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Nuclear factor TDP-43 and SR proteins promote in vitro and in vivo CFTR exon 9 skipping
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Self-citationMutations in progranulin are a major cause of ubiquitin-positive frontotemporal lobar degeneration
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Self-citationTDP-43 Binds Heterogeneous Nuclear Ribonucleoprotein A/B through Its C-terminal Tail
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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
Molecular Mechanisms of TDP-43 Misfolding and Pathology in Amyotrophic Lateral Sclerosis
Prasad, A., Bharathi, V., Sivalingam, V., Girdhar, A., & Patel, B. K. (2019) · Frontiers in Molecular Neuroscience · doi:10.3389/fnmol.2019.00025 · Cited by 774
TDP-43 mutant transgenic mice develop features of ALS and frontotemporal lobar degeneration
Wegorzewska, I., Bell, S., Cairns, N. J., Miller, T. M., & Baloh, R. H. (2009) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0908767106 · Cited by 706
Gains or losses: molecular mechanisms of TDP43-mediated neurodegeneration
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Mackenzie, I. R., Nicholson, A. M., Sarkar, M., Messing, J., Purice, M. D., Pottier, C., Annu, K., Baker, M., Perkerson, R. B., Kurti, A., et al. (2017) · Neuron · doi:10.1016/j.neuron.2017.07.025 · Cited by 640
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Metadata
- Authors
- Zhang, Y.-J., Xu, Y.-F., Dickey, C. A., Buratti, E., Baralle, F., Bailey, R., Pickering-Brown, S., Dickson, D., & Petrucelli, L.
- Venue
- The Journal of Neuroscience
- Publisher
- Society for Neuroscience
- Published
- 2007-09-26
- Volume
- 27
- Issue
- 39
- Pages
- 10530-10534
- ISSN
- 0270-6474 (ISSN-L)
- 1529-2401
- OpenAlex
- W2169092675
- Cited by
- 371
- References
- 16
- FWCI
- 19.86
- Type
- journal article
- Language
- en
- Open access
- hybrid
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- Zhang, Y.-J., Xu, Y.-F., Dickey, C. A., Buratti, E., Baralle, F., Bailey, R., Pickering-Brown, S., Dickson, D., & Petrucelli, L. (2007). Progranulin Mediates Caspase-Dependent Cleavage of TAR DNA Binding Protein-43. The Journal of Neuroscience. https://doi.org/10.1523/jneurosci.3421-07.2007
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