The DOI matches its Crossref record; 7 of the 7 references it cites were found in a registry or an index.
TDP-43 transgenic mice develop spastic paralysis and neuronal inclusions characteristic of ALS and frontotemporal lobar degeneration
Open accessAbstract
Neuronal cytoplasmic and intranuclear aggregates of RNA-binding protein TDP-43 are a hallmark feature of neurodegenerative diseases such as amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD). ALS and FTLD show a considerable clinical and pathological overlap and occur as both familial and sporadic forms. Though missense mutations in TDP-43 cause rare forms of familial ALS, it is not yet known whether this is due to loss of TDP-43 function or gain of aberrant function. Moreover, the role of wild-type (WT) TDP-43, associated with the majority of familial and sporadic ALS/FTLD patients, is also currently unknown. Generating homozygous and hemizygous WT human TDP-43 transgenic mouse lines, we show here a dose-dependent degeneration of cortical and spinal motor neurons and development of spastic quadriplegia reminiscent of ALS. A dose-dependent degeneration of nonmotor cortical and subcortical neurons characteristic of FTLD was also observed. Neurons in the affected spinal cord and brain regions showed accumulation of TDP-43 nuclear and cytoplasmic aggregates that were both ubiquitinated and phosphorylated as observed in ALS/FTLD patients. Moreover, the characteristic ≈25-kDa C-terminal fragments (CTFs) were also recovered from nuclear fractions and correlated with disease development and progression in WT TDP-43 mice. These findings suggest that ≈25-kDa TDP-43 CTFs are noxious to neurons by a gain of aberrant nuclear function.
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Cites
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Cited by
Converging Mechanisms in ALS and FTD: Disrupted RNA and Protein Homeostasis
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TDP-43 and FUS/TLS: emerging roles in RNA processing and neurodegeneration
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The changing scene of amyotrophic lateral sclerosis
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TDP-43 proteinopathy and motor neuron disease in chronic traumatic encephalopathy
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Metadata
- Authors
- Wils, H., Kleinberger, G., Janssens, J., Pereson, S., Joris, G., Cuijt, I., Smits, V., Ceuterick-de Groote, C., Van Broeckhoven, C., & Kumar-Singh, S.
- Venue
- Proceedings of the National Academy of Sciences
- Published
- 2010-02-23
- Volume
- 107
- Issue
- 8
- Pages
- 3858-3863
- ISSN
- 0027-8424 (ISSN-L)
- 1091-6490
- OpenAlex
- W2070086306
- Cited by
- 587
- References
- 7
- FWCI
- 34.04
- Type
- journal article
- Language
- en
- Open access
- green
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- Wils, H., Kleinberger, G., Janssens, J., Pereson, S., Joris, G., Cuijt, I., Smits, V., Ceuterick-de Groote, C., Van Broeckhoven, C., & Kumar-Singh, S. (2010). TDP-43 transgenic mice develop spastic paralysis and neuronal inclusions characteristic of ALS and frontotemporal lobar degeneration. Proceedings of the National Academy of Sciences. https://doi.org/10.1073/pnas.0912417107
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