From clinical description, to in vitro and animal studies, and backward to patients: Oxidative stress and mitochondrial dysfunction in Fanconi anemia

► Oxidative stress (OS) has multiple roles in the Fanconi anemia (FA) phenotype. ► We review in vitro mitochondrial dysfunction (MDF) in FA cells. ► There is a need to elucidate MDF vs OS endpoints in vivo in FA patients. ► The occurrence of nitrosative stress in FA is being investigated. ► We should move toward chemoprevention trials aimed at offsetting MDF with “mitochondrial nutrients.”