These two drugs are safe when administered in humans; cysteamine is used to treat cystinosis (33, 34), and both cysteamine and cystamine have been used in human clinical trials in the treatment of diseases which directly or indirectly implicate TG2 and autophagy deregulation such as Huntington disease (35), cystic fibrosis (36, 37), and celiac disease (38). This evidence concerns the gene TGM2 and juvenile Huntington disease.