Modelling amyotrophic lateral sclerosis in rodents.

Area of Science:

• Neuroscience
• Genetics
• Pathology

Background:

• Accurate animal models are essential for studying human diseases like amyotrophic lateral sclerosis (ALS).
• Most existing ALS animal models are based on familial genetic mutations, despite sporadic cases being more common.
• The discovery of new ALS-associated genes necessitates the development of corresponding animal models.

Purpose of the Study:

• To provide a comprehensive review of all amyotrophic lateral sclerosis (ALS) rodent models developed to date.
• To highlight the importance of rodent models in verifying genetic variant pathogenicity and understanding disease mechanisms.
• To emphasize the role of these models in preclinical therapeutic testing for ALS.

Main Methods:

• Systematic literature review of published research on amyotrophic lateral sclerosis (ALS) rodent models.
• Categorization and analysis of existing genetic and spontaneous rodent models of ALS.
• Evaluation of the utility of these models in recapitulating human ALS pathology and progression.

Main Results:

• A wide array of rodent models for amyotrophic lateral sclerosis (ALS) has been developed, reflecting various genetic mutations and spontaneous pathologies.
• These models offer valuable platforms for studying the central nervous system (CNS) aspects of ALS.
• The models facilitate the validation of novel genetic findings and provide insights into disease pathogenesis.

Conclusions:

• Rodent models are indispensable tools for advancing amyotrophic lateral sclerosis (ALS) research.
• The continued development and characterization of ALS rodent models are critical for understanding disease mechanisms and accelerating therapeutic discovery.
• This review serves as a resource for researchers selecting appropriate models for their specific ALS studies.