Amyotrophic Lateral Sclerosis (ALS) is a deadly neurodegenerative disease characterized by a precipitous loss of motor neuron function. From the time researchers first named the disease, the key protein that either causes ALS or accumulates because of the disease remained a mystery.
Then, in 2006, a research team led by University of Pennsylvania neuroscientists found TAR DNA-binding protein 43 (TDP-43) lurking in the protinaceous inclusions that spider through the neurons of many ALS patients, as described in this month's Hot Paper.
The research team initially was searching for the protein causing frontotemporal lobar degeneration with ubiquinated inclusions (FTLD-U) - a dementia-causing disorder related to ALS. The team screened mouse antibodies against nearly 50,000 samples of diseased brain tissue from FTLD-U patients, and found that TDP-43 was the key pathologic protein in ...
