Medical research is crucial to expanding an understanding of certain conditions, which ultimately leads to improved diagnostic and treatment options, and more social awareness. In a recent press release, CurePSP announced its intentions to expand research around progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD). The organization is doing so by awarding three Venture Grants of $100,000 to researchers who will explore how tau protein pathology and genetics play a role in these two conditions.

CurePSP Research Grants

For 31 years, nonprofit advocacy organization CurePSP has worked to raise awareness around a series of neurodegenerative diseases. Outside of PSP and CBD, CurePSP also provides education around multiple system atrophy (MSA). In addition to raising awareness and providing educational resources, CurePSP also hopes to improve patient care and discover potential treatment options. Now, the organization will contribute to this research through the Venture Grants.

Altogether, three researchers received Venture Grants. These include:

Dr. Rohan de Silva

A researcher at UCL Queen Square Institute of Neurology in London, Dr. de Silva was given the grant to learn more about how tau protein spreads. Normally, tau helps to stabilize neuronal microtubules. But in patients with PSP and CBD, tau becomes toxic and causes neuronal damage. In his research, Dr. de Silva hopes to understand how and why this tau goes rogue. Additionally, he hopes to learn whether the specific structural forms of toxic tau could act as biomarkers or create more targeted therapies.

Dr. Franziska Hopfner

Dr. Hopfner hails from the Department of Neurology at Hannover Medical School in Germany. Her research focuses on the relationship between epigenetics and toxic tau accumulation. Could epigenetic neuronal changes cause over-accumulation of toxic tau? In particular, Dr. Hopfner will explore microRNA. Ultimately, her goal is to determine whether or not this research can help to develop new therapeutic options for patients with PSP or CBD.

Dr. Chao Peng

The final grant was awarded to Dr. Chao Peng of the Department of Neurology at the UCLA David Geffen School of Medicine. In Dr. Peng’s work, he will explore how modulating healthy tau protein could inhibit toxic tau from spreading. To do so, Dr. Peng will utilize autopsied brains sourced from patients with PSP. Through his research, he hopes to develop a novel treatment to stop or partially inhibit disease progression. 

Altogether, CurePSP awards Venture Grants twice each year. Interested in applying? The next deadline is May 1, 2021. Apply here today.

Progressive Supranuclear Palsy (PSP)

Gradual nerve cell damage in the brain stem causes progressive supranuclear palsy (PSP), although the cause of that damage is still somewhat unknown. In rare cases, MAPT gene mutations cause PSP. However, in other cases, all researchers know is that patients with PSP present with abnormal toxic tau accumulations in the brain. This progressive neurological disorder affects movement and speech. In fact, it is aptly named: progressive (worsening), palsy (causes weakness), and supranuclear (affecting parts of the brain above nerve cells).

An estimated 3-6 in every 100,000 individuals has PSP. The condition affects males more than females. Often, PSP symptom onset occurs between ages 45-75. Currently, there is no cure, although some treatments are available to mitigate symptoms. These symptoms include:

• Difficulty eating or swallowing
• Muscle stiffness
• Light sensitivity
• Depression, anxiety, or apathy
• Progressively worsening balance, including difficulty walking and frequent backward falls
• Slowed movement
• Impulsivity
• Difficulty with problem-solving, memory, or judgment
• Sleep disturbances
• Slurred, slowed, or quiet speech
• Difficulty looking downward
• Blurred or double vision

Corticobasal Degeneration (CBD)

While medical professionals are unaware of the exact cause of corticobasal degeneration (CBD), many believe the condition results from a mixture of genetics, aging, and environmental factors. CBD is a rare and progressive neurological which causes cell loss, damage, and deterioration throughout the brain. Specifically, CBD impacts the areas of the brain responsible for movement and information processes. As a result, patients with CBD experience a variety of cognitive, behavioral, and motor-related symptoms which vary in severity. Typically, CBD symptoms affect one limb initially. Later, as the disease progresses, the other limbs are affected. Patients with CBD are at an increased risk of developing serious and sometimes life-threatening infections. 

Symptoms include:

• Tremors or muscle spasms 
• Slowed movement (bradykinesia)
• Difficulty swallowing
• Muscle rigidity and stiffness, particularly affecting the limbs
• Poor balance and coordination
• Dystonia (involuntary muscle contractions)
• Changes in gait
• Abnormal eye movements
• Difficulty expressing or understanding language