Neurodegeneration in amyotrophic lateral sclerosis: The role of oxidative stress and altered homeostasis of metals

Maria Teresa Carrì, Alberto Ferri, Mauro Cozzolino, Lilia Calabrese, Giuseppe Rotilio

Research output: Contribution to journalArticle

147 Citations (Scopus)

Abstract

Amyotrophic lateral sclerosis is one of the most common neurodegenerative disorders, with an incidence of about 1/100,000. One of the typical features of this progressive, lethal disease, occurring both sporadically and as a familial disorder, is degeneration of cortical and spinal motor neurones. Present evidence indicates that loss of neurones in patients results from a complex interplay among oxidative injury, excitotoxic stimulation, dysfunction of critical proteins and genetic factors. This review focuses on existing evidence that oxidative stress is a major culprit in the pathogenesis of amyotrophic lateral sclerosis. An increase in reactive oxygen species and in products of oxidation has been observed both in post-mortem samples and in experimental models for ALS. This increase may be consequent to altered metabolism of copper and iron ions, that share the property to undergo redox cycling and generate reactive oxygen species. Metal-mediated oxidative stress would lead to several intracellular alterations and contribute to the induction of cell death pathways.

Original languageEnglish
Pages (from-to)365-374
Number of pages10
JournalBrain Research Bulletin
Volume61
Issue number4
DOIs
Publication statusPublished - Aug 30 2003

Fingerprint

Amyotrophic Lateral Sclerosis
Reactive Oxygen Species
Oxidative Stress
Homeostasis
Metals
Motor Neurons
Neurodegenerative Diseases
Oxidation-Reduction
Copper
Cell Death
Theoretical Models
Iron
Ions
Neurons
Incidence
Wounds and Injuries
Proteins

Keywords

  • Amyotrophic lateral sclerosis
  • Copper
  • Iron
  • Neurodegeneration
  • Oxidative stress
  • Superoxide dismutase

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Neurodegeneration in amyotrophic lateral sclerosis : The role of oxidative stress and altered homeostasis of metals. / Carrì, Maria Teresa; Ferri, Alberto; Cozzolino, Mauro; Calabrese, Lilia; Rotilio, Giuseppe.

In: Brain Research Bulletin, Vol. 61, No. 4, 30.08.2003, p. 365-374.

Research output: Contribution to journalArticle

@article{3f09a771dbd14b119b7fc1857d2ca9cf,
title = "Neurodegeneration in amyotrophic lateral sclerosis: The role of oxidative stress and altered homeostasis of metals",
abstract = "Amyotrophic lateral sclerosis is one of the most common neurodegenerative disorders, with an incidence of about 1/100,000. One of the typical features of this progressive, lethal disease, occurring both sporadically and as a familial disorder, is degeneration of cortical and spinal motor neurones. Present evidence indicates that loss of neurones in patients results from a complex interplay among oxidative injury, excitotoxic stimulation, dysfunction of critical proteins and genetic factors. This review focuses on existing evidence that oxidative stress is a major culprit in the pathogenesis of amyotrophic lateral sclerosis. An increase in reactive oxygen species and in products of oxidation has been observed both in post-mortem samples and in experimental models for ALS. This increase may be consequent to altered metabolism of copper and iron ions, that share the property to undergo redox cycling and generate reactive oxygen species. Metal-mediated oxidative stress would lead to several intracellular alterations and contribute to the induction of cell death pathways.",
keywords = "Amyotrophic lateral sclerosis, Copper, Iron, Neurodegeneration, Oxidative stress, Superoxide dismutase",
author = "Carr{\`i}, {Maria Teresa} and Alberto Ferri and Mauro Cozzolino and Lilia Calabrese and Giuseppe Rotilio",
year = "2003",
month = "8",
day = "30",
doi = "10.1016/S0361-9230(03)00179-5",
language = "English",
volume = "61",
pages = "365--374",
journal = "Brain Research Bulletin",
issn = "0361-9230",
publisher = "Elsevier Inc.",
number = "4",

}

TY - JOUR

T1 - Neurodegeneration in amyotrophic lateral sclerosis

T2 - The role of oxidative stress and altered homeostasis of metals

AU - Carrì, Maria Teresa

AU - Ferri, Alberto

AU - Cozzolino, Mauro

AU - Calabrese, Lilia

AU - Rotilio, Giuseppe

PY - 2003/8/30

Y1 - 2003/8/30

N2 - Amyotrophic lateral sclerosis is one of the most common neurodegenerative disorders, with an incidence of about 1/100,000. One of the typical features of this progressive, lethal disease, occurring both sporadically and as a familial disorder, is degeneration of cortical and spinal motor neurones. Present evidence indicates that loss of neurones in patients results from a complex interplay among oxidative injury, excitotoxic stimulation, dysfunction of critical proteins and genetic factors. This review focuses on existing evidence that oxidative stress is a major culprit in the pathogenesis of amyotrophic lateral sclerosis. An increase in reactive oxygen species and in products of oxidation has been observed both in post-mortem samples and in experimental models for ALS. This increase may be consequent to altered metabolism of copper and iron ions, that share the property to undergo redox cycling and generate reactive oxygen species. Metal-mediated oxidative stress would lead to several intracellular alterations and contribute to the induction of cell death pathways.

AB - Amyotrophic lateral sclerosis is one of the most common neurodegenerative disorders, with an incidence of about 1/100,000. One of the typical features of this progressive, lethal disease, occurring both sporadically and as a familial disorder, is degeneration of cortical and spinal motor neurones. Present evidence indicates that loss of neurones in patients results from a complex interplay among oxidative injury, excitotoxic stimulation, dysfunction of critical proteins and genetic factors. This review focuses on existing evidence that oxidative stress is a major culprit in the pathogenesis of amyotrophic lateral sclerosis. An increase in reactive oxygen species and in products of oxidation has been observed both in post-mortem samples and in experimental models for ALS. This increase may be consequent to altered metabolism of copper and iron ions, that share the property to undergo redox cycling and generate reactive oxygen species. Metal-mediated oxidative stress would lead to several intracellular alterations and contribute to the induction of cell death pathways.

KW - Amyotrophic lateral sclerosis

KW - Copper

KW - Iron

KW - Neurodegeneration

KW - Oxidative stress

KW - Superoxide dismutase

UR - http://www.scopus.com/inward/record.url?scp=0042165931&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0042165931&partnerID=8YFLogxK

U2 - 10.1016/S0361-9230(03)00179-5

DO - 10.1016/S0361-9230(03)00179-5

M3 - Article

C2 - 12909279

AN - SCOPUS:0042165931

VL - 61

SP - 365

EP - 374

JO - Brain Research Bulletin

JF - Brain Research Bulletin

SN - 0361-9230

IS - 4

ER -