Disturbance of redox homeostasis in Down Syndrome: Role of iron dysmetabolism

Eugenio Barone, Andrea Arena, Elizabeth Head, D. Allan Butterfield, Marzia Perluigi

Research output: Contribution to journalReview articlepeer-review

40 Scopus citations

Abstract

Down Syndrome (DS) is the most common genetic form of intellectual disability that leads in the majority of cases to development of early-onset Alzheimer-like dementia (AD). The neuropathology of DS has several common features with AD including alteration of redox homeostasis, mitochondrial deficits, and inflammation among others. Interestingly, some of the genes encoded by chromosome 21 are responsible of increased oxidative stress (OS) conditions that are further exacerbated by decreased antioxidant defense. Previous studies from our groups showed that accumulation of oxidative damage is an early event in DS neurodegeneration and that oxidative modifications of selected proteins affects the integrity of the protein degradative systems, antioxidant response, neuronal integrity and energy metabolism. In particular, the current review elaborates recent findings demonstrating the accumulation of oxidative damage in DS and we focus attention on specific deregulation of iron metabolism, which affects both the central nervous system and the periphery. Iron dysmetabolism is a well-recognized factor that contributes to neurodegeneration; thus we opine that better understanding how and to what extent the concerted loss of iron dyshomeostasis and increased OS occur in DS could provide novel insights for the development of therapeutic strategies for the treatment of Alzheimer-like dementia.

Original languageEnglish
Pages (from-to)84-93
Number of pages10
JournalFree Radical Biology and Medicine
Volume114
DOIs
StatePublished - Jan 2018

Bibliographical note

Publisher Copyright:
© 2017 Elsevier Inc.

Keywords

  • Iron
  • Oxidative stress
  • Protein oxidation
  • Redox proteomics
  • Trisomy 21

ASJC Scopus subject areas

  • Biochemistry
  • Physiology (medical)

Fingerprint

Dive into the research topics of 'Disturbance of redox homeostasis in Down Syndrome: Role of iron dysmetabolism'. Together they form a unique fingerprint.

Cite this