RT Journal A1 Kuehn BM T1 NEw model system offers clues to als JF JAMA JO JAMA YR 2011 FD October 12 VO 306 IS 14 SP 1534 OP 1534 DO 10.1001/jama.2011.1439 UL http://dx.doi.org/10.1001/jama.2011.1439 AB ALS is a progressive and ultimately fatal disorder in which the motor neurons in a patient's central nervous system die off, causing paralysis and eventually respiratory failure. Most cases are sporadic, but about 20% are linked to mutations in the superoxide dismutase 1 (SOD1) gene. However, scientists have so far been unable to identify the precise mechanisms behind the motor neuron loss.