Dementia

Toxic interaction in neurons that leads to dementia and ALS

December 12, 2012

Theoretical model illustrating how misregulation of human SORT1 splicing affects PGRN levels (credit: Mercedes Prudencio et al./PNAS)

Researchers at Mayo Clinic in Florida haveuncovered a toxic cellular process by which a protein that maintains the health of neurons becomes deficient and can lead to dementia.

The findings shed new light on the link between culprits implicated in two devastating neurological diseases: Alzheimer’s disease and amyotrophic lateral sclerosis, also known as Lou Gehrig’s disease, which afflicts physicist Stephen Hawking.

There is no cure for frontotemporal dementia, a disorder that affects personality, behavior and language and is second only to Alzheimer’s disease as the most common form of early-onset dementia.