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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Self-citationMutations in LRRK2 Cause Autosomal-Dominant Parkinsonism with Pleomorphic Pathology
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Mutations in progranulin cause tau-negative frontotemporal dementia linked to chromosome 17
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Self-citationProgressive Supranuclear Palsy
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Tau is a candidate gene for chromosome 17 frontotemporal dementia
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Tau A152T risk variant drives distinct cell type transcriptional signatures in AD and PSP
Lilley, M. T., Ren, Y., Disha, S., Sanborn, E. C., Marks, J. D., Chen, Y., Yan, Y., Deture, M. A., Rawlinson, B. D., Dickson, D. W., et al. (2026) · Acta Neuropathologica · doi:10.1007/s00401-026-03110-6
Self-citationThe characteristics of perivascular proinflammation in 4‐repeat tauopathies
Fu, M., Uruk, G., Gatto, R. G., Reichard, R. R., Whitwell, J. L., & Josephs, K. A. (2026) · Brain Pathology · doi:10.1111/bpa.70162
Molecular Mechanisms of Frontotemporal Lobar Degeneration
Skoglund, L. (2009) · KTH Publication Database DiVA (KTH Royal Institute of Technology)
Hu, Q. (2012) · Research Explorer (The University of Manchester)
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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