humoral immunity is partly intact and it’s possible that cytokines are released by B cells or neutrophils. Currently, the administration of ALS is basically signs based, and riluzole, a representative, is Afatinib EGFR inhibitor the only drug for the treatment of ALS accepted by the food and drug administration. Objective: We reviewed current literature concerning promising therapies for amyotrophic lateral sclerosis. Methods: A Medline literature search was conducted to identify all studies on ALS treatment printed from January 1st, 1986 through August 31st, 2009. Papers were selected by us concerning only disease modifying therapy. Results: 48 compounds were identified and reviewed in this study. Conclusions: Riluzole is the only substance that demonstrated a beneficial impact on ALS patients, but with only moderate increase in survival. Even though many drugs showed results in the animal models for ALS, not one of them significantly prolonged survival or improved quality of life of ALS patients. Many factors have been implicated Cellular differentiation in explaining the mostly negative effects of numerous randomized clinical trials in ALS, including methodological issues in the use of animal drug testing, the shortage of evaluation of pharmacokinetic profile of the drugs, and methodological issues of clinical trials in ALS patients. Amyotrophic lateral sclerosis is really a somewhat rare neurodegenerative disorder characterized by progressive lack of both upper and lower motor neurons in the back, brainstem, and brain. The development of the condition is normally rapid, ultimately causing death on average within 3 C5 years. 1 The fundamental cause of ALS remains unclear, but an interaction between endogenous and exogenous factors is believed to be involved with the growth of the disease. 2,3 Although ALS often develops occasionally, five hundred C10% of cases are familial and heritable. Thirty % of familial ALS are brought on by the mutation in Cu/Zn superoxide dismutase 1 gene. 1 The development of animal models of ALS has order Docetaxel presented progress in understanding the fundamental mechanisms of the disease as the irregular and the common kinds of ALS discuss comparable clinical and pathological features. 3,4 A few animal models have been extensively found in ALS in recent times, including different transgenic mouse models, wobbler mouse and one canine model. 3,4 The most clinically appropriate animal model of ALS is the SOD1 transgenic rodent model, that is genetically engineered to express a mutant type of the human SOD1 gene. The most popular SOD1 mouse harbors the glycine to alanine mutation at position 93. This mutation results in a harmful gain of function of Cu/Zn SOD1 that promotes the generation of harmful oxygen radicals.