Development of Nanostructured Lipid Carriers for the Delivery of Idebenone in Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay.

ABSTRACT: Oxidative stress occurs when physiological antioxidant systems do not manage to counteract the excessive intracellular production of reactive oxygen species (ROS), which accumulate leading to irreversible oxidation of DNA and other biomacromolecules, and thus to the onset of pathological conditions. Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is a neurodegenerative disease characterized by autosomal recessive mutations in the sacsin gene (SACS). It has been demonstrated that cells of ARSACS patients show bioenergetic and mitochondrial impairment, denoted by reduced respiratory chain activities and ATP synthesis. In order to design a suitable therapy for ARSACS, it is essential to consider that treatments need to cross the blood-brain barrier (BBB), a specializ

SUBMITTER: Martinelli C 

PROVIDER: S-EPMC7271403 | biostudies-literature | 2020 Jun

REPOSITORIES: biostudies-literature