Recent progress in the discovery of small molecules for the treatment of amyotrophic lateral sclerosis (ALS)

• Allison S. Limpert,
• Margrith E. Mattmann and
• Nicholas D. P. Cosford

Beilstein J. Org. Chem. 2013, 9, 717–732, doi:10.3762/bjoc.9.82

• preclinical characterization of lead compounds that may ultimately provide novel drugs to treat patients suffering from ALS. Keywords: amyotrophic lateral sclerosis (ALS); copper/zinc (Cu-Zn) superoxide dismutase 1 (SOD1); glutamate toxicity; neurodegeneration; oxidative stress; Introduction Amyotrophic

• (sALS) and 10% of all cases characterized as familial (fALS) [4]. Several gene mutations have been identified that contribute to this disorder with 20% of fALS cases being linked to mutations in the copper/zinc (Cu-Zn) superoxide dismutase 1 (SOD1) gene [4]. Many cellular pathologies have been