Neurology Central

Build-up of RNA-binding proteins found in individuals with ALS and dementia

Researchers from Columbia University (NY, USA) and the New York Genome Center (NY, USA) have demonstrated that RNA-binding proteins appear to accumulate in the brain in the most common versions of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Previously, it was understood that this build-up occurred only in the less common, hereditary forms of these diseases.

Inherited forms of the neurodegenerative diseases ALS and FTD have been linked to genetic mutations that lead to the build-up of RNA-binding protein TDP-43. Previous studies have reported that a mutation can lead to a fragment of DNA repeating itself more than would be expected. RNA binding proteins – such as TDP-43 or hnRNP H – attach to these repeats, leading to the degeneration of brain cells.

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