The DOI matches its Crossref record; 21 of the 21 references it cites were found in a registry or an index.
TAR DNA-binding protein 43 in neurodegenerative disease
Open accessAbstract
In 2006, TAR DNA-binding protein 43 (TDP-43), a highly conserved nuclear protein, was identified as the major disease protein in amyotrophic lateral sclerosis (ALS) and in the most common variant of frontotemporal lobar degeneration (FTLD), FTLD-U, which is characterized by cytoplasmic inclusions that stain positive for ubiquitin but negative for tau and α-synuclein. Since then, rapid advances have been made in our understanding of the physiological function of TDP-43 and the role of this protein in neurodegeneration. These advances link ALS and FTLD-U (now designated FTLD-TDP) to a shared mechanism of disease. In this Review, we summarize the current evidence regarding the normal function of TDP-43 and the TDP-43 pathology observed in FTLD-TDP, ALS, and other neurodegenerative diseases wherein TDP-43 pathology co-occurs with other disease-specific lesions (for example, with amyloid plaques and neurofibrillary tangles in Alzheimer disease). Moreover, we discuss the accumulating data that support our view that FTLD-TDP and ALS represent two ends of a spectrum of primary TDP-43 proteinopathies. Finally, we comment on the importance of recent advances in TDP-43-related research to neurological practice, including the new opportunities to develop better diagnostics and disease-modifying therapies for ALS, FTLD-TDP, and related disorders exhibiting TDP-43 pathology.
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Cites
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Current state of Alzheimer’s fluid biomarkers
Molinuevo, J. L., Ayton, S., Batrla, R., Bednar, M. M., Bittner, T., Cummings, J., Fagan, A. M., Hampel, H., Mielke, M. M., Mikulskis, A., et al. (2018) · Acta Neuropathologica · doi:10.1007/s00401-018-1932-x · Cited by 518
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Metadata
- Authors
- Chen-Plotkin, A. S., Lee, V. M.-Y., & Trojanowski, J. Q.
- Venue
- Nature Reviews Neurology
- Published
- 2010-03-16
- Volume
- 6
- Issue
- 4
- Pages
- 211-220
- ISSN
- 1759-4758 (ISSN-L)
- 1759-4766
- OpenAlex
- W2051582477
- Cited by
- 468
- References
- 21
- FWCI
- 4.28
- Type
- journal article
- Language
- en
- Open access
- green
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