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 language | English |
|---|---|
| Article number | 510 |
| Journal | Frontiers in Neuroscience |
| Volume | 12 |
| Issue number | AUG |
| DOIs | |
| Publication status | Published - Aug 7 2018 |
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Keywords
- ALS
- Channels
- Ionic homeostasis
- Neurodegeneration
- Transporters
ASJC Scopus subject areas
- Neuroscience(all)
Cite this
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 journal › Review article
}
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 -