Ionic homeostasis maintenance in ALS: Focus on new therapeutic targets

Rossana Sirabella, Valeria Valsecchi, Serenella Anzilotti, Ornella Cuomo, Antonio Vinciguerra, Pasquale Cepparulo, Paola Brancaccio, Natascia Guida, Nicolas Blondeau, Lorella M.T. Canzoniero, Cristina Franco, Salvatore Amoroso, Lucio Annunziato, Giuseppe Pignataro

Research output: Contribution to journalReview article

7 Citations (Scopus)

Abstract

Amyotrophic lateral sclerosis (ALS) is one of the most threatening neurodegenerative disease since it causes muscular paralysis for the loss of Motor Neurons in the spinal cord, brainstem and motor cortex. Up until now, no effective pharmacological treatment is available. Two forms of ALS have been described so far: 90% of the cases presents the sporadic form (sALS) whereas the remaining 10% of the cases displays the familiar form (fALS). Approximately 20% of fALS is associated with inherited mutations in the Cu, Zn-superoxide dismutase 1 (SOD1) gene. In the last decade, ionic homeostasis dysregulation has been proposed as the main trigger of the pathological cascade that brings to motor-neurons loss. In the light of these premises, the present review will analyze the involvement in ALS pathophysiology of the most well studied metal ions, i.e., calcium, sodium, iron, copper and zinc, with particular focus to the role of ionic channels and transporters able to contribute in the regulation of ionic homeostasis, in order to propose new putative molecular targets for future therapeutic strategies to ameliorate the progression of this devastating neurodegenerative disease.

Original languageEnglish
Article number510
JournalFrontiers in Neuroscience
Volume12
Issue numberAUG
DOIs
Publication statusPublished - Aug 7 2018

Fingerprint

Amyotrophic Lateral Sclerosis
Homeostasis
Maintenance
Motor Neurons
Neurodegenerative Diseases
Motor Cortex
Ion Channels
Paralysis
Brain Stem
Zinc
Copper
Spinal Cord
Therapeutics
Iron
Metals
Sodium
Pharmacology
Ions
Calcium
Mutation

Keywords

  • ALS
  • Channels
  • Ionic homeostasis
  • Neurodegeneration
  • Transporters

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Sirabella, R., Valsecchi, V., Anzilotti, S., Cuomo, O., Vinciguerra, A., Cepparulo, P., ... Pignataro, G. (2018). Ionic homeostasis maintenance in ALS: Focus on new therapeutic targets. Frontiers in Neuroscience, 12(AUG), [510]. https://doi.org/10.3389/fnins.2018.00510

Ionic homeostasis maintenance in ALS : Focus on new therapeutic targets. / Sirabella, Rossana; Valsecchi, Valeria; Anzilotti, Serenella; Cuomo, Ornella; Vinciguerra, Antonio; Cepparulo, Pasquale; Brancaccio, Paola; Guida, Natascia; Blondeau, Nicolas; Canzoniero, Lorella M.T.; Franco, Cristina; Amoroso, Salvatore; Annunziato, Lucio; Pignataro, Giuseppe.

In: Frontiers in Neuroscience, Vol. 12, No. AUG, 510, 07.08.2018.

Research output: Contribution to journalReview article

Sirabella, R, Valsecchi, V, Anzilotti, S, Cuomo, O, Vinciguerra, A, Cepparulo, P, Brancaccio, P, Guida, N, Blondeau, N, Canzoniero, LMT, Franco, C, Amoroso, S, Annunziato, L & Pignataro, G 2018, 'Ionic homeostasis maintenance in ALS: Focus on new therapeutic targets', Frontiers in Neuroscience, vol. 12, no. AUG, 510. https://doi.org/10.3389/fnins.2018.00510
Sirabella, Rossana ; Valsecchi, Valeria ; Anzilotti, Serenella ; Cuomo, Ornella ; Vinciguerra, Antonio ; Cepparulo, Pasquale ; Brancaccio, Paola ; Guida, Natascia ; Blondeau, Nicolas ; Canzoniero, Lorella M.T. ; Franco, Cristina ; Amoroso, Salvatore ; Annunziato, Lucio ; Pignataro, Giuseppe. / Ionic homeostasis maintenance in ALS : Focus on new therapeutic targets. In: Frontiers in Neuroscience. 2018 ; Vol. 12, No. AUG.
@article{e8eb1cbd32b64d3a9c080a95a6f00307,
title = "Ionic homeostasis maintenance in ALS: Focus on new therapeutic targets",
abstract = "Amyotrophic lateral sclerosis (ALS) is one of the most threatening neurodegenerative disease since it causes muscular paralysis for the loss of Motor Neurons in the spinal cord, brainstem and motor cortex. Up until now, no effective pharmacological treatment is available. Two forms of ALS have been described so far: 90{\%} of the cases presents the sporadic form (sALS) whereas the remaining 10{\%} of the cases displays the familiar form (fALS). Approximately 20{\%} of fALS is associated with inherited mutations in the Cu, Zn-superoxide dismutase 1 (SOD1) gene. In the last decade, ionic homeostasis dysregulation has been proposed as the main trigger of the pathological cascade that brings to motor-neurons loss. In the light of these premises, the present review will analyze the involvement in ALS pathophysiology of the most well studied metal ions, i.e., calcium, sodium, iron, copper and zinc, with particular focus to the role of ionic channels and transporters able to contribute in the regulation of ionic homeostasis, in order to propose new putative molecular targets for future therapeutic strategies to ameliorate the progression of this devastating neurodegenerative disease.",
keywords = "ALS, Channels, Ionic homeostasis, Neurodegeneration, Transporters",
author = "Rossana Sirabella and Valeria Valsecchi and Serenella Anzilotti and Ornella Cuomo and Antonio Vinciguerra and Pasquale Cepparulo and Paola Brancaccio and Natascia Guida and Nicolas Blondeau and Canzoniero, {Lorella M.T.} and Cristina Franco and Salvatore Amoroso and Lucio Annunziato and Giuseppe Pignataro",
year = "2018",
month = "8",
day = "7",
doi = "10.3389/fnins.2018.00510",
language = "English",
volume = "12",
journal = "Frontiers in Neuroscience",
issn = "1662-4548",
publisher = "Frontiers Media S.A.",
number = "AUG",

}

TY - JOUR

T1 - Ionic homeostasis maintenance in ALS

T2 - Focus on new therapeutic targets

AU - Sirabella, Rossana

AU - Valsecchi, Valeria

AU - Anzilotti, Serenella

AU - Cuomo, Ornella

AU - Vinciguerra, Antonio

AU - Cepparulo, Pasquale

AU - Brancaccio, Paola

AU - Guida, Natascia

AU - Blondeau, Nicolas

AU - Canzoniero, Lorella M.T.

AU - Franco, Cristina

AU - Amoroso, Salvatore

AU - Annunziato, Lucio

AU - Pignataro, Giuseppe

PY - 2018/8/7

Y1 - 2018/8/7

N2 - Amyotrophic lateral sclerosis (ALS) is one of the most threatening neurodegenerative disease since it causes muscular paralysis for the loss of Motor Neurons in the spinal cord, brainstem and motor cortex. Up until now, no effective pharmacological treatment is available. Two forms of ALS have been described so far: 90% of the cases presents the sporadic form (sALS) whereas the remaining 10% of the cases displays the familiar form (fALS). Approximately 20% of fALS is associated with inherited mutations in the Cu, Zn-superoxide dismutase 1 (SOD1) gene. In the last decade, ionic homeostasis dysregulation has been proposed as the main trigger of the pathological cascade that brings to motor-neurons loss. In the light of these premises, the present review will analyze the involvement in ALS pathophysiology of the most well studied metal ions, i.e., calcium, sodium, iron, copper and zinc, with particular focus to the role of ionic channels and transporters able to contribute in the regulation of ionic homeostasis, in order to propose new putative molecular targets for future therapeutic strategies to ameliorate the progression of this devastating neurodegenerative disease.

AB - Amyotrophic lateral sclerosis (ALS) is one of the most threatening neurodegenerative disease since it causes muscular paralysis for the loss of Motor Neurons in the spinal cord, brainstem and motor cortex. Up until now, no effective pharmacological treatment is available. Two forms of ALS have been described so far: 90% of the cases presents the sporadic form (sALS) whereas the remaining 10% of the cases displays the familiar form (fALS). Approximately 20% of fALS is associated with inherited mutations in the Cu, Zn-superoxide dismutase 1 (SOD1) gene. In the last decade, ionic homeostasis dysregulation has been proposed as the main trigger of the pathological cascade that brings to motor-neurons loss. In the light of these premises, the present review will analyze the involvement in ALS pathophysiology of the most well studied metal ions, i.e., calcium, sodium, iron, copper and zinc, with particular focus to the role of ionic channels and transporters able to contribute in the regulation of ionic homeostasis, in order to propose new putative molecular targets for future therapeutic strategies to ameliorate the progression of this devastating neurodegenerative disease.

KW - ALS

KW - Channels

KW - Ionic homeostasis

KW - Neurodegeneration

KW - Transporters

UR - http://www.scopus.com/inward/record.url?scp=85053635450&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85053635450&partnerID=8YFLogxK

U2 - 10.3389/fnins.2018.00510

DO - 10.3389/fnins.2018.00510

M3 - Review article

AN - SCOPUS:85053635450

VL - 12

JO - Frontiers in Neuroscience

JF - Frontiers in Neuroscience

SN - 1662-4548

IS - AUG

M1 - 510

ER -