Litcius/Paper detail

Cell Rearrangement and Oxidant/Antioxidant Imbalance in Huntington’s Disease

Francesco D’Egidio, Vanessa Castelli, Annamaria Cimini, Michele d’Angelo

2023Antioxidants40 citationsDOIOpen Access PDF

Abstract

gene, resulting in the production of an aberrant huntingtin (Htt) protein. The mutant protein accumulation is responsible for neuronal dysfunction and cell death. This is due to the involvement of oxidative damage, excitotoxicity, inflammation, and mitochondrial impairment. Neurons naturally adapt to bioenergetic alteration and oxidative stress in physiological conditions. However, this dynamic system is compromised when a neurodegenerative disorder occurs, resulting in changes in metabolism, alteration in calcium signaling, and impaired substrates transport. Thus, the aim of this review is to provide an overview of the cell's answer to the stress induced by HD, focusing on the role of oxidative stress and its balance with the antioxidant system.

Topics & Concepts

HuntingtinExcitotoxicityHuntington's diseaseOxidative stressNeuroprotectionHuntingtin ProteinNeurodegenerationCell biologyProgrammed cell deathBiologyAntioxidantBioenergeticsOxidative phosphorylationApoptosisNeuroscienceMitochondrionChemistryDiseaseBiochemistryInternal medicineMedicineGenetic Neurodegenerative DiseasesMitochondrial Function and PathologyMuscle Physiology and Disorders