Even though neurodegenerative diseases often strike in middle age or later, patients could have structural differences in their brains that arise before birth.
and reduces the size of two key regions of the brain during embryonic development.
Up to 10% of cases of ALS and FTD result from mutations in a gene called C9ORF72, even though the two diseases usually manifest very differently. Whereas ALS, also known as Lou Gehrig's disease, causes progressive paralysis and death within three to five years of diagnosis, FTD most commonly affects behavior, personality and language.
Compared to neural stem cells derived from healthy people, the neural stem cells from patients with ALS or FTD weren't able to renew their population. Instead, the patient-derived neural stem cells had a tendency to prematurely differentiate into mature neurons. The scientists wanted to understand the consequences of a non-renewing population of neural stem cells in a living organism, so they turned toIn embryonic mice, mutations in C9ORF72 caused measurable developmental changes not only in their brains, but also elsewhere in their bodies. In their brains, these changes included smaller thalamic regions, as well as reduced cortical thickness. The mice also weighed 5–10% less at embryonic day 18.5.
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