Columbia University Medical Center (CUMC) researchers have identified a gene, called matrix metalloproteinase - 9 (MMP - 9), that appears to play a major role in
motor neuron degeneration in amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease.
For three years now we have been working on human cell models of rare neurodegenerative diseases with special emphasis on neuroacanthocytosis, neuronal ceroid lipofuscinosis as well
as motor neuron degeneration (using iPS cells).
The article examines the onset of motor neuron loss in a mouse model of SMA and sheds light on the pathophysiological mechanisms
of motor neuron degeneration.