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6792334 
Journal Article 
Therapeutic neuroprotective agents for amyotrophic lateral sclerosis 
Pandya, RS; Zhu, H; Li, Wei; Bowser, R; Friedlander, RM; Wang, Xin; , 
2013 
Yes 
Cellular and Molecular Life Sciences (CMLS)
ISSN: 1420-682X
EISSN: 1420-9071 
SPRINGER BASEL AG 
BASEL 
4729-4745 
23864030 
WOS:000327095100007 
Amyotrophic lateral sclerosis (ALS) is a fatal chronic neurodegenerative disease whose hallmark is proteinaceous, ubiquitinated, cytoplasmic inclusions in motor neurons and surrounding cells. Multiple mechanisms proposed as responsible for ALS pathogenesis include dysfunction of protein degradation, glutamate excitotoxicity, mitochondrial dysfunction, apoptosis, oxidative stress, and inflammation. It is therefore essential to gain a better understanding of the underlying disease etiology and search for neuroprotective agents that might delay disease onset, slow progression, prolong survival, and ultimately reduce the burden of disease. Because riluzole, the only Food and Drug Administration (FDA)-approved treatment, prolongs the ALS patient's life by only 3 months, new therapeutic agents are urgently needed. In this review, we focus on studies of various small pharmacological compounds targeting the proposed pathogenic mechanisms of ALS and discuss their impact on disease progression.