The DOI matches its Crossref record; 99 of the 99 references it cites were found in a registry or an index.
Progressive Supranuclear Palsy: Pathology and Genetics
Open accessAbstract
Progressive supranuclear palsy (PSP) is an atypical Parkinsonian disorder associated with progressive axial rigidity, vertical gaze palsy, dysarthria and dysphagia. Neuropathologically, the subthalamic nucleus and brainstem, especially the midbrain tectum and the superior cerebellar peduncle, show atrophy. The substantia nigra shows loss of pigment corresponding to nigrostriatal dopaminergic degeneration. Microscopic findings include neuronal loss, gliosis and neurofibrillary tangles in basal ganglia, diencephalon and brainstem. Characteristic tau pathology is also found in glia. The major genetic risk factor for sporadic PSP is a common variant in the gene encoding microtubule‐associated protein tau (MAPT) and recent studies have suggested that this may result in the altered expression of specific tau protein isoforms. Imaging studies suggest that there may be sensitive and specific means to differentiate PSP from other parkinsonian disorders, but identification of a diagnostic biomarker is still elusive.
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“Progressive supranuclear palsy (PSP) is a devastating neurodegenerative disorder caused by the neuro-glial aggregation of tau protein, particularly in the basal ganglia, diencephalon, and brainstem1,2.”
From Neuroinflammation and Tau Colocalize in vivo in Progressive Supranuclear Palsy (2020) · cites this paper as [1]
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Association of missense and 5′-splice-site mutations in tau with the inherited dementia FTDP-17
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Self-citationMutations in LRRK2 Cause Autosomal-Dominant Parkinsonism with Pleomorphic Pathology
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Cloning of the Gene Containing Mutations that Cause PARK8-Linked Parkinson's Disease
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Mutations in progranulin cause tau-negative frontotemporal dementia linked to chromosome 17
Baker, M., Mackenzie, I. R., Pickering-Brown, S. M., Gass, J., Rademakers, R., Lindholm, C., Snowden, J., Adamson, J., Sadovnick, A. D., Rollinson, S., et al. (2006) · Nature · doi:10.1038/nature05016 · Cited by 2,058
Self-citationProgressive Supranuclear Palsy
Steele, J. C. (1964) · Archives of Neurology · doi:10.1001/archneur.1964.00460160003001 · Cited by 1,930
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Self-citationMutation in the tau gene in familial multiple system tauopathy with presenile dementia
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Tau is a candidate gene for chromosome 17 frontotemporal dementia
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A common inversion under selection in Europeans
Stefánsson, H., Helgason, A., Þorleifsson, G., Steinthórsdóttir, V., Másson, G., Barnard, J., Baker, A., Jónasdóttir, Á., Ingason, A., Gudnadottir, V. G., et al. (2005) · Nature Genetics · doi:10.1038/ng1508 · Cited by 847
Cited by
Sorted by citations
Parkinson's Disease and Parkinsonism: Neuropathology
Dickson, D. W. (2012) · Cold Spring Harbor Perspectives in Medicine · doi:10.1101/cshperspect.a009258 · Cited by 847
Self-citationParkinson's Disease and Parkinsonism
Hayes, M. T. (2019) · The American Journal of Medicine · doi:10.1016/j.amjmed.2019.03.001 · Cited by 823
Arendt, T., Stieler, J. T., & Holzer, M. (2016) · Brain Research Bulletin · doi:10.1016/j.brainresbull.2016.08.018 · Cited by 609
Identification of common variants influencing risk of the tauopathy progressive supranuclear palsy
PSP Genetics Study Group, Höglinger, G. U., Melhem, N. M., Dickson, D. W., Sleiman, P. M. A., Wang, L.-S., Klei, L., Rademakers, R., de Silva, R., Litvan, I., et al. (2011) · Nature Genetics · doi:10.1038/ng.859 · Cited by 603
Self-citationInvited review: Neuropathology of tauopathies: principles and practice
Kovacs, G. G. (2015) · Neuropathology and Applied Neurobiology · doi:10.1111/nan.12208 · Cited by 492
Metadata
- Authors
- Dickson, D. W., Rademakers, R., & Hutton, M. L.
- Affiliations
- Mayo Clinic in Florida (US)
- Venue
- Brain Pathology
- Publisher
- Wiley
- Published
- 2007-01
- Volume
- 17
- Issue
- 1
- Pages
- 74-82
- ISSN
- 1015-6305 (ISSN-L)
- 1750-3639
- PMC
- PMC8095545
- OpenAlex
- W2014875179
- Cited by
- 314
- References
- 99
- Citations by year
- 2026: 20 · 2025: 24 · 2024: 25 · 2023: 25 · 2022: 15 · 2021: 17 · 2020: 11 · 2019: 18 · 2018: 7 · 2017: 25 · 2016: 19 · 2015: 11 · 2014: 17 · 2013: 14 · 2012: 10
- FWCI
- 1.58
- Type
- journal article
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Locations
- Brain Pathology (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- Funders
- National Institute on Aging (R01AG015866, P01AG014449, P01AG003949, P50AG025711, P01AG017216, P50AG016574)
- National Institute of Neurological Disorders and Stroke (P01NS040256, P50NS040256)
- National Institute of Environmental Health Sciences (R01ES013941, R01-ES02941)
- Topics
- Health Sciences › Medicine › Clinical Neurology › Parkinson's Disease Mechanisms and Treatments
- Health Sciences › Medicine › Clinical Neurology › Deep Brain Stimulation and Movement Disorders
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Alzheimer's disease research and treatments
- Keywords
- progressive supranuclear palsy, tauopathy, tau pathology, MAPT gene, tau isoforms, atypical parkinsonism, subthalamic nucleus, midbrain tectum, superior cerebellar peduncle, nigrostriatal degeneration, neurofibrillary tangles, gliosis, brainstem atrophy, differential diagnosis, diagnostic biomarkers, neuroimaging
- MeSH terms
- Humans, Supranuclear Palsy, Progressive, tau Proteins
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