title: Could Sirtuin Activities Modify ALS Onset and Progression?
abstract: Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease with a complex etiology. Sirtuins have been implicated as disease-modifying factors in several neurological disorders, and in the past decade, attempts have been made to check if manipulating Sirtuin activities and levels could confer benefit in terms of neuroprotection and survival in ALS models. The efforts have largely focused on mutant SOD1, and while limited in scope, the results were largely positive. Here, the body of work linking Sirtuins with ALS is reviewed, with discussions on how Sirtuins and their activities may impact on the major etiological mechanisms of ALS. Moving forward, it is important that the potentially beneficial effect of Sirtuins in ALS disease onset and progression are assessed in ALS models with TDP-43, FUS, and C9orf72 mutations.
authors.affiliation: Department of Biochemistry, Yong Loo Lin School of Medicine, National University of Singapore, MD7, 8 Medical Drive, Singapore, 117597, Singapore. bchtbl@nus.edu.sg.|NUS Graduate School for Integrative Sciences and Engineering, National University of Singapore, 28 Medical Drive, Singapore, 117456, Singapore. bchtbl@nus.edu.sg.
authors.forename: Bor Luen
authors.initials: BL
authors.lastname: Tang
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000188:drug therapy / Q000378:metabolism* / Q000473:pathology*; D000818:Animals; D018450:Disease Progression; D006801:Humans; D009046:Motor Neurons / Q000187:drug effects / Q000378:metabolism / Q000473:pathology; D037761:Sirtuins / Q000378:metabolism* / Q000627:therapeutic use*; D000072105:Superoxide Dismutase-1 / Q000378:metabolism
chemical_list: D000072105:Superoxide Dismutase-1; D037761:Sirtuins
keywords: Amyotrophic lateral sclerosis (ALS); Motor neuron; Neuronal degeneration; Sirtuins