Journal
CELLULAR AND MOLECULAR LIFE SCIENCES
Volume 78, Issue 17-18, Pages 6143-6160Publisher
SPRINGER BASEL AG
DOI: 10.1007/s00018-021-03905-8
Keywords
Experimental animal models; Genetic modifiers; Neuroprotection; Therapeutics
Categories
Funding
- Italian Ministry of Health (Ricerca Corrente)
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ALS is a rare and devastating disease involving genetic, cellular, and molecular mechanisms, with Drosophila research playing a significant role in understanding its pathogenesis and potential therapeutic strategies. This review summarizes the progress and challenges in studying ALS modifiers with Drosophila models.
Amyotrophic lateral sclerosis (ALS) is a rare, devastating disease, causing movement impairment, respiratory failure and ultimate death. A plethora of genetic, cellular and molecular mechanisms are involved in ALS signature, although the initiating causes and progressive pathological events are far from being understood. Drosophila research has produced seminal discoveries for more than a century and has been successfully used in the past 25 years to untangle the process of ALS pathogenesis, and recognize potential markers and novel strategies for therapeutic solutions. This review will provide an updated view of several ALS modifiers validated in C9ORF72, SOD1, FUS, TDP-43 and Ataxin-2 Drosophila models. We will discuss basic and preclinical findings, illustrating recent developments and novel breakthroughs, also depicting unsettled challenges and limitations in the Drosophila-ALS field. We intend to stimulate a renewed debate on Drosophila as a screening route to identify more successful disease modifiers and neuroprotective agents.
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