Редактирование: ASIC2 (раздел)

==Publications==

{{medline-entry
|title=Acidotoxicity and acid-sensing ion channels contribute to motoneuron degeneration.
|pubmed-url=https://pubmed.ncbi.nlm.nih.gov/23306556
|abstract=Amyotrophic lateral sclerosis (ALS) is a fatal neurological condition with no cure. Mitochondrial dysfunction, Ca(2 ) overloading and local hypoxic/ischemic environments have been implicated in the pathophysiology of ALS and are conditions that may initiate metabolic acidosis in the affected tissue. We tested the hypothesis that acidotoxicity and acid-sensing ion channels (ASICs) are involved in the pathophysiology of ALS. We found that motoneurons were selectively vulnerable to acidotoxicity in vitro, and that acidotoxicity was partially reduced in asic1a-deficient motoneuron cultures. Cross-breeding of [[SOD1]](G93A) ALS mice with asic1a-deficient mice delayed the onset and progression of motor dysfunction in [[SOD1]] mice. Interestingly, we also noted a strong increase in [[ASIC2]] expression in motoneurons of [[SOD1]] mice and sporadic ALS patients during disease progression. Pharmacological pan-inhibition of ASIC channels with the lipophilic amiloride derivative, 5-(N,N-dimethyl)-amiloride hydrochloride, potently protected cultured motoneurons against acidotoxicity, and, given post-symptom onset, significantly improved lifespan, motor performance and motoneuron survival in [[SOD1]] mice. Together, our data provide strong evidence for the involvement of acidotoxicity and ASIC channels in motoneuron degeneration, and highlight the potential of ASIC inhibitors as a new treatment approach for ALS.
|mesh-terms=* Acid Sensing Ion Channels
* Acids
* Amiloride
* Amyotrophic Lateral Sclerosis
* Animals
* Apoptosis
* Cells, Cultured
* Disease Models, Animal
* Humans
* Longevity
* Male
* Mice
* Mice, Inbred C57BL
* Mice, Knockout
* Mice, Transgenic
* Motor Neurons
* Mutation
* Superoxide Dismutase
* Superoxide Dismutase-1

|full-text-url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3595486
}}