What is ALS?
Amyotrophic Lateral Sclerosis (ALS), also known as “Lou Gehrig’s Disease,” is a progressive neurodegenerative disease. The degeneration of motor neurons in the brain and spinal cord undermines their ability to send impulses to muscle fibers which normally lead to muscle movement. This leads to the diminishment of the ability of the brain to control muscle movement. As such, there is a progressive degeneration of voluntary muscle action, which can lead to paralysis. Early signs of ALS may include muscle weakness of the arms and legs, along with difficulty speaking, breathing and swallowing. Muscles eventually atrophy, or decrease in size.
Importantly, ALS involves the diminishment of voluntary movements. While motor neurons are involved in the functioning of heart and digestive muscles, these actions are involuntary and as such are not affected by ALS. The cause of ALS is not yet fully understood, but significant progress is being made. The overall incidence of ALS is two per 100,000 people, with an average life expectancy of two to five years following diagnosis.
Key Research Publications on Amyotrophic Lateral Sclerosis:
Jung MK, Kim KY, Lee NY, Kang YS, Hwang YJ, Kim Y, Sung JJ, McKee A, Kowall N, Lee J, Ryu H. Expression of Taurine Transporter (TauT) is Modulated by Heat Shock Factor 1 (HSF1) in Motor Neurons of ALS. Mol Neurobiol. 2012 Nov 23. [PDF]
Factor-Litvak P, Al-Chalabi A, Ascherio A, Bradley W, Chío A, Garruto R, Hardiman O, Kamel F, Kasarskis E, McKee A, Nakano I, Nelson LM, Eisen A. Current pathways for epidemiological research in amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener. 2013 May;14 Suppl 1:33-43. [PDF]
Lee JK, Shin JH, Hwang SG, Gwag BJ, McKee AC, Lee J, Kowall NW, Ryu H, Lim DS, Choi EJ. MST1 functions as a key modulator of neurodegeneration in a mouse model of ALS. Proc Natl Acad Sci U S A. 2013 Jul 1. [PDF]