Could Conservative Iron Chelation Lead to Neuroprotection in Amyotrophic Lateral Sclerosis?

ABSTRACT: Iron accumulation has been observed in mouse models and in both sporadic and familial forms of amyotrophic lateral sclerosis (ALS). Iron chelation could reduce iron accumulation and the related excess of oxidative stress in the motor pathways. However, classical iron chelation would induce systemic iron depletion. We assess the safety and efficacy of conservative iron chelation (i.e., chelation with low risk of iron depletion) in a murine preclinical model and pilot clinical trial. In Sod1^G86R mice, deferiprone increased the mean life span compared with placebo. The safety was good, without anemia after 12 months of deferiprone in the 23 ALS patients enrolled in the clinical trial. The decreases in the ALS Functional Rating Scale and the body mass index were significantly smalle

SUBMITTER: Moreau C 

PROVIDER: S-EPMC6067092 | biostudies-literature | 2018 Sep

REPOSITORIES: biostudies-literature