Abnormal energy metabolism in ALS: a key player?

ABSTRACT:

Purpose of the review

Amyotrophic lateral sclerosis (ALS) is an adult-onset neurodegenerative disease of the motor system due to the selective and progressive degeneration of both upper and lower motor neurons. Disturbances in energy homeostasis were repeatedly associated with the ALS pathogenesis and appear early during the disease process. In this review, we highlight recent work demonstrating the crucial role of energy metabolism in ALS and discuss its potential clinical relevance.

Recent findings

The alteration of various metabolic pathways contributes to the heterogeneity of the clinical phenotype of ALS. Recent work showed that different ALS mutations selectively impact these pathways and translate to the disease phenotypes in patients and disease models. Strikingly, a

SUBMITTER: Burg T 

PROVIDER: S-EPMC10328537 | biostudies-literature | 2023 Aug

REPOSITORIES: biostudies-literature