“Brain Donations: Who, What, Where, When, and Why?” Webinar
Friday, March 17, 2023
Co-hosts: The Association for Frontotemporal Degeneration (AFTD) and the FTD Disorders Registry
Dr. Larry Golbe, a world-renowned expert on progressive supranuclear palsy (PSP), has his own blog (psp-blog.org) to which he occasionally publishes insights into the latest research.
In late March 2022, he published about two papers he read on the protein TMEM106B. He says, “This stuff is known to be a component of healthy lysosomes and endosomes, components of the cell’s garbage disposal mechanism.”[One paper] found that the brains of healthy elderly persons have abnormal aggregates of a misfolded form of the protein TMEM106B. These abnormal aggregates were found “even more abundantly in a raft of neurodegenerative diseases: Alzheimer’s, CBD, multiple types of FTD, Parkinson’s, dementia with Lewy bodies, multiple system atrophy and multiple sclerosis.”
The second paper (authors from Columbia University, Mayo Clinic Jacksonville, etc) “found the same TMEM106B aggregates” in those with PSP.
Dr. Golbe says,
An interesting finding is that unlike tau, TMEM106B misfolds the same way in all the diseases analyzed so far. This may have huge potential implications: if (and this is a big “if”) the misfolded TMEM106B plays an important role in the formation of the misfolding and toxicity of tau and the other disease-specific proteins, and if (another big “if”) this misfolding is the rate-limiting step in the loss of brain cells in the neurodegenerative disorders, THEN preventing TMEM106B from forming or from misfolding, or targeting it with antibodies or drugs could be the silver bullet that prevents all of these diseases, PSP included. That could be a naïve hope…
Read Dr. Golbe’s blog post:
“Common thread, silver bullet, naïve hope?”
Dr. Larry Golbe
March 16, 2022
This terrific article from the Wall Street Journal in the fall of 2021 notes:
“As the number of people with dementia grows, more of them are speaking out to challenge assumptions about what they can and can’t do. A group of advocates, many in the earlier stages of this condition, say that people around them often struggle with understanding the full range of symptoms. … Life expectancy for those with early-onset dementia varies. One 2019 study showed a mean survival time of 17 years after symptoms start and 10 years after a diagnosis.”
In this newspaper article about a new, large Dutch study, frontotemporal dementia (FTD) and Lewy body dementia (LBD) are both described is common causes of young-onset dementia. Continue reading