Mar 22 2024Picower Institute at MIT On the surface, the movement disorder amyotrophic lateral sclerosis , also known as Lou Gehrig's disease, and the cognitive disorder frontotemporal lobar degeneration , which underlies frontotemporal dementia, manifest in very different ways. In addition, they are known to primarily affect very different regions of the brain.
Manolis Kellis, co-senior author of the paper and professor in the Computer Science and Artificial Intelligence Laboratory at MIT Another key realization from the study is that brain donors with inherited vs. sporadic forms of the disease showed similarly altered gene expression changes, even though these were previously thought to have different causes. That suggests that similar molecular processes could be going awry downstream of the diseases' origins.
Related Stories"UMNs and spindle neurons look nothing alike and live in very different areas of the brain" said Sebastian Pineda, lead author of the study, and a graduate student jointly supervised by Heiman and Kellis. "It was remarkable to see that they appear practically indistinguishable at the molecular level and respond very similarly to disease."
The study also found that the most vulnerable cells expressed genes known to be genetically-associated with each disease, providing a potential mechanistic basis for some of these genetic associations. This pattern is not always the case in neurodegenerative conditions, Heiman said.