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Basic Science and Pathogenesis.

Belay Gebregergis1,2, Liam T Ralph2, Liang Zhang1,3

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Summary
This summary is machine-generated.

C9orf72 deficiency causes synaptic hyperexcitability and excitotoxicity in frontotemporal dementia models. Targeting calcium-permeable AMPA receptors (CP-AMPARs) offers a potential therapeutic strategy for C9orf72-associated neurodegeneration.

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Area of Science:

  • Neuroscience
  • Genetics
  • Cell Biology

Background:

  • C9orf72 gene expansions are the leading genetic cause of frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS).
  • C9orf72 deficiency is linked to synaptic dysfunction and neurodegeneration, with hyperexcitability and excitotoxicity as emerging hallmarks.
  • The precise mechanisms underlying these deficits in dementia pathology are not fully understood.

Purpose of the Study:

  • To investigate the role of C9orf72 in synaptic function and excitotoxic vulnerability using a mouse model.
  • To explore the involvement of calcium-permeable AMPA receptors (CP-AMPARs) in C9orf72-associated neurodegeneration.
  • To evaluate the therapeutic potential of CP-AMPAR antagonists.

Main Methods:

  • Utilized C9orf72-knockout (C9-KO) mice to model the disease.
  • Assessed hippocampal synaptic markers, dendritic spine morphology, and CP-AMPAR-mediated plasticity.
  • Induced excitotoxic stress with kainic acid (KA) to analyze seizure susceptibility, network stability (EEG), and GluA1 expression, followed by CP-AMPAR antagonist treatment.

Main Results:

  • C9-KO mice showed increased synaptic hyperexcitability, evidenced by elevated surface GluA1, reduced dendritic spine density, and enlarged spine heads.
  • These mice exhibited enhanced CP-AMPAR plasticity and greater vulnerability to KA-induced excitotoxicity, including severe seizures and abnormal EEG.
  • Selective CP-AMPAR antagonism successfully mitigated excitotoxic damage and restored synaptic function.

Conclusions:

  • C9orf72 deficiency exacerbates synaptic hyperexcitability and excitotoxic vulnerability via CP-AMPAR dysregulation, contributing to FTD pathophysiology.
  • Loss of C9orf72 amplifies excitatory signaling, linking synaptic dysfunction to network instability and neurodegeneration.
  • CP-AMPARs are identified as key mediators of excitotoxicity, representing a promising therapeutic target for C9orf72-related dementias and other neurodegenerative disorders.