Common Protein Defect Linked to Lou Gehrig's Disease and Frontotemporal Dementia

Researchers at the University of Pennsylvania School of Medicine have identified a protein abnormality that appears central to both Lou Gehrig's disease (ALS) and frontotemporal dementia (FTD), two fatal and currently incurable neurological conditions.

Post-mortem examination of brain tissue from 72 patients who had suffered from either disorder revealed an excess buildup of a protein called TDP-43 in all cases, the scientists reported.

The team noted that where the toxic protein accumulates determines which disease develops: deposits in the frontal and temporal lobes, which govern judgment and behavior, are associated with FTD, while buildup in spinal cord motor neurons, which control movement, is linked to ALS.

Scientists had long suspected a biological connection between the two disorders, and the findings suggest TDP-43 misfolding is the shared pathological link. The study appeared in the 6 October 2006 issue of the journal Science.

Historical summary. TurkishPress restated this wire report, first published in October 2006, in its own words.