Progressive Supranuclear Palsy

About PSP and TauC3

PSP is a rare and fatal degenerative neurological disorder affecting about 20,000 people in the United States.  It causes progressive impairment of balance and walking; impaired eye movement, abnormal muscle tone, speech difficulties, and problems related to swallowing and eating. Affected individuals also frequently experience personality changes and cognitive impairment. Symptoms typically begin after age 60 but can begin earlier. The exact cause of PSP is unknown, and the disease is often initially misdiagnosed as Parkinson’s disease. No disease-modifying treatments have been approved for either the early or late disease stages.

In PSP, tauC3 production and activity have been linked to a single polynuclear polymorphism at rs1768208, a significant risk factor for the disease (Figure 4).  Thus, TBL-100 could be beneficial for this condition as well.

Figure 4.In this model, increasing appoptosin expression driven by a PSP risk allele triggers a caspase cascade that promotes synaptic dysfunction and neurodegeneration through cleavage of tau. From Appoptosin-Mediated Caspase Cleavage of Tau Contributed to Progressive Supranuclear Palsy Pathogenesis.