Progressive Supranuclear Palsy (PSP)
Progressive supranuclear palsy (PSP) is a rare neurodegenerative disorder characterized by the accumulation of abnormal tau protein in the brain. At Protheragen, we are committed to advancing the understanding and management of PSP through cutting-edge therapeutic development and disease modeling services. Our goal is to provide end-to-end solutions for the entire process from PSP therapeutic research to commercialization.
Overview of Progressive Supranuclear Palsy (PSP)
Progressive supranuclear palsy (PSP) is a rare, neurodegenerative tauopathy characterized by progressive deterioration of motor function, oculomotor deficits, and cognitive impairment. The incidence of PSP is approximately 0.02-0.83 cases per 10,000 population. First described by Steele, Richardson, and Olszewski in 1964, PSP remains a challenging disorder with no disease-modifying therapies currently available.
Fig.1 Summary of the differential diagnosis of rare progressive supranuclear palsy (PSP) subtypes. (Krzosek P, et al., 2022)
Pathogenesis of Progressive Supranuclear Palsy (PSP)
Progressive supranuclear palsy (PSP) is classified as a rare neurodegenerative disorder that fall under tauopathies, defined by the accumulation of hyperphosphorylated 4-repeat tau protein in neurons and glial cells, especially in the basal ganglia, brainstem, and frontal cortex. It is known that a strong association exists between the disease and H1 haplotype of MAPT gene which drives the aggregation of tau into neurofibrillary tangles and tufted astrocytes. Such changes are responsible for the selective degeneration of important neuronal cell groups.
Fig.2 Tau hyperphosphorylation in progressive supranuclear palsy (PSP). (Ichikawa-Escamilla E, et al., 2024)
Therapeutic Development for Progressive Supranuclear Palsy (PSP)
| Drug Names | Mechanism of Action | Targets | NCT Number | Research Phase |
| RT001 | Synthetic deuterated polyunsaturated fatty acid (D-PUFA) that reduces lipid peroxidation and oxidative stress in neurons | Lipid membranes | NCT04937530 | Phase II |
| GV1001 | Telomerase-derived peptide with neuroprotective and anti-inflammatory effects; may reduce tau aggregation | Tau protein | NCT06235775 | Phase II |
| FNP-223 | Small molecule tau aggregation inhibitor; prevents pathological tau fibrillization | 4R tau oligomers/fibrils | NCT06355531 | Phase II |
| TPN-101 | Selective kinase inhibitor modulating tau phosphorylation and neuroinflammation | Tau kinases | NCT04993768 | Phase II |
Disclaimer: Protheragen focuses on providing preclinical research services. This table is for information exchange purposes only. This table is not a treatment plan recommendation. For guidance on treatment options, please visit a regular hospital.
Our Services
Protheragen focuses on the complete progressive supranuclear palsy (PSP) solutions by combining early disease detection with advanced in vitro diagnostic (IVD) kits and developing diagnostics. Our therapeutic development pipeline uses proprietary disease models like physiologically representative blood-brain barrier (BBB) models to accelerate investigation and validation of treatments targeting the central nervous system (CNS).
Therapeutic Development Services
By Mechanism of Action
Disease Model Development Services
In Vitro Model
Development
Development
- P301L Transgenic Model
- A152T Transgenic Model
- hTau-40/P301L Rat Model
- Tau35 Overexpression Model
- Diphteria-urotensin II Neurotoxin Induction Model
- Other Models
At Protheragen, we are committed to validating and optimizing therapies for progressive supranuclear palsy (PSP) through preclinical studies including pharmacodynamics (PD), pharmacokinetics (PK) and toxicology to ensure their successful regulatory approval. If you are interested in our services, please feel free to contact us for more details and quotation information of related services.
References
- Krzosek P, Madetko N, Migda A, et al. Differential diagnosis of rare subtypes of progressive supranuclear palsy and PSP-like syndromes—infrequent manifestations of the most common form of atypical parkinsonism[J]. Frontiers in Aging Neuroscience, 2022, 14: 804385.
- Ichikawa-Escamilla E, Velasco-Martínez R A, Adalid-Peralta L. Progressive supranuclear palsy syndrome: An overview[J]. IBRO Neuroscience Reports, 2024.