Cited by 91Parkinson's Disease Mechanisms and TreatmentsAlzheimer's disease research and treatmentsNeurological disorders and treatments

Rational therapeutic approaches to progressive supranuclear palsy

Stamelou, M., de Silva, R., Arias-Carrion, O., Boura, E., Hollerhage, M., Oertel, W. H., Muller, U., Hoglinger, G. U.·2010·Brain

Abstract

Progressive supranuclear palsy is a sporadic and progressive neurodegenerative disease, most often presenting as a symmetric, akinetic-rigid syndrome with postural instability, vertical supranuclear gaze palsy and frontal lobe deficits. It belongs to the family of tauopathies and involves both cortical and subcortical structures. Although the exact pathophysiology is not yet fully understood, several lines of evidence point to a crucial contribution from both genetic predisposition and mitochondrial dysfunction. Recently gained insights into the pathophysiology of this disease have led to several hypothesis-driven therapeutic approaches aiming at disease-modification rather than mere symptomatic neurotransmitter-replacement therapy. Agents targeting mitochondrial dysfunction have already shown a positive effect in a phase II study and further studies to verify and expand these results are ongoing. Clinical studies with agents targeting tau dysfunction such as tau-kinase inhibitors, tau-aggregation inhibitors and microtubule stabilizers are in preparation or ongoing. This review presents the current pathophysiological concepts driving these exciting therapeutic developments.

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Registry

Crossref · 10.1093/brain/awq115

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Authors
Stamelou, M., de Silva, R., Arias-Carrion, O., Boura, E., Hollerhage, M., Oertel, W. H., Muller, U., & Hoglinger, G. U.
Affiliations
Philipps University of Marburg (DE)
Venue
Brain
Published
2010-05-14
Volume
133
Issue
6
Pages
1578-1590
ISSN
  • 0006-8950 (ISSN-L)
  • 1460-2156
OpenAlex
W2043797799
Cited by
91
References
160
Citations by year
2026: 2 · 2025: 5 · 2024: 4 · 2023: 1 · 2021: 5 · 2020: 4 · 2019: 7 · 2018: 4 · 2017: 9 · 2016: 9 · 2015: 7 · 2014: 16 · 2013: 6 · 2012: 4
FWCI
0.68
Type
journal article
Language
en
Open access
closed
Locations
  • Brain (publishedVersion)
  • PubMed (publishedVersion)
  • UCL Discovery (University College London) (submittedVersion)
Funders
  • CurePSP
  • Deutsche Forschungsgemeinschaft (HO2402/6-1)
  • Medical Research Council (G0501560)
Topics
  • Health Sciences › Medicine › Neurology › Parkinson's Disease Mechanisms and Treatments
  • Health Sciences › Medicine › Physiology › Alzheimer's disease research and treatments
  • Health Sciences › Medicine › Neurology › Neurological disorders and treatments
Keywords
progressive supranuclear palsy, tauopathy, mitochondrial dysfunction, disease-modifying therapy, tau aggregation inhibitors, microtubule stabilizers, akinetic-rigid syndrome, postural instability, genetic susceptibility, neurodegeneration
MeSH terms
Animals, Humans, Mitochondria, Supranuclear Palsy, Progressive

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