The DOI matches its Crossref record; 4 of the 4 references it cites were found in a registry or an index.
ALS motor phenotype heterogeneity, focality, and spread: Deconstructing motor neuron degeneration
Open accessAbstract
Heterogeneity of motor phenotypes is a clinically well-recognized fundamental aspect of amyotrophic lateral sclerosis (ALS) and is determined by variability of 3 independent primary attributes: body region of onset; relative mix of upper motor neuron (UMN) and lower motor neuron (LMN) deficits; and rate of progression. Motor phenotypes are determined by the anatomy of the underlying neuropathology and the common defining elements underlying their heterogeneity are that motor neuron degeneration is fundamentally a focal process and that it spreads contiguously through the 3-dimensional anatomy of the UMN and LMN levels, thus causing seemingly complex and varied clinical manifestations. This suggests motor neuron degeneration in ALS is in actuality a very orderly and actively propagating process and that fundamental molecular mechanisms may be uniform and their chief properties deduced. This also suggests opportunities for translational research to seek pathobiology directly in the less affected regions of the nervous system.
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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
Astrocytes expressing ALS-linked mutated SOD1 release factors selectively toxic to motor neurons
Nagai, M., Re, D. B., Nagata, T., Chalazonitis, A., Jessell, T. M., Wichterle, H., & Przedborski, S. (2007) · Nature Neuroscience · doi:10.1038/nn1876 · Cited by 1,220
Neurovascular signalling defects in neurodegeneration
Zacchigna, S., Lambrechts, D., & Carmeliet, P. (2008) · Nature Reviews Neuroscience · doi:10.1038/nrn2336 · Cited by 345
Cited by
Decoding ALS: from genes to mechanism
Taylor, J. P., Brown, R. H., & Cleveland, D. W. (2016) · Nature · doi:10.1038/nature20413 · Cited by 2,066
Self-propagation of pathogenic protein aggregates in neurodegenerative diseases
Jucker, M., & Walker, L. C. (2013) · Nature · doi:10.1038/nature12481 · Cited by 1,663
van Es, M. A., Hardiman, O., Chio, A., Al-Chalabi, A., Pasterkamp, R. J., Veldink, J. H., & van den Berg, L. H. (2017) · The Lancet · doi:10.1016/s0140-6736(17)31287-4 · Cited by 1,298
Amyotrophic lateral sclerosis: a clinical review
Masrori, P., & Van Damme, P. (2020) · European Journal of Neurology · doi:10.1111/ene.14393 · Cited by 1,201
Stages of pTDP‐43 pathology in amyotrophic lateral sclerosis
Brettschneider, J., Del Tredici, K., Toledo, J. B., Robinson, J. L., Irwin, D. J., Grossman, M., Suh, E., Van Deerlin, V. M., Wood, E. M., Baek, Y., et al. (2013) · Annals of Neurology · doi:10.1002/ana.23937 · Cited by 1,065
Metadata
- Authors
- Ravits, J. M., & La Spada, A. R.
- Venue
- Neurology
- Published
- 2009-09-08
- Volume
- 73
- Issue
- 10
- Pages
- 805-811
- ISSN
- 0028-3878 (ISSN-L)
- 1526-632X
- OpenAlex
- W2157557701
- Cited by
- 604
- References
- 4
- FWCI
- 16.31
- Type
- journal article
- Language
- en
- Open access
- bronze
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- Ravits, J. M., & La Spada, A. R. (2009). ALS motor phenotype heterogeneity, focality, and spread: Deconstructing motor neuron degeneration. Neurology. https://doi.org/10.1212/wnl.0b013e3181b6bbbd
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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.
