Neural circuit and synaptic dysfunctions in ALS-FTD pathology

Santiago Mora, Ilary Allodi*

*Corresponding author for this work

Research output: Contribution to journalReviewResearchpeer-review

8 Citations (Scopus)
23 Downloads (Pure)

Abstract

Action selection is a capital feature of cognition that guides behavior in processes that range from motor patterns to executive functions. Here, the ongoing actions need to be monitored and adjusted in response to sensory stimuli to increase the chances of reaching the goal. As higher hierarchical processes, these functions rely on complex neural circuits, and connective loops found within the brain and the spinal cord. Successful execution of motor behaviors depends, first, on proper selection of actions, and second, on implementation of motor commands. Thus, pathological conditions crucially affecting the integrity and preservation of these circuits and their connectivity will heavily impact goal-oriented motor behaviors. Amyotrophic Lateral Sclerosis (ALS) and Frontotemporal Dementia (FTD) are two neurodegenerative disorders known to share disease etiology and pathophysiology. New evidence in the field of ALS-FTD has shown degeneration of specific neural circuits and alterations in synaptic connectivity, contributing to neuronal degeneration, which leads to the impairment of motor commands and executive functions. This evidence is based on studies performed on animal models of disease, post-mortem tissue, and patient derived stem cells. In the present work, we review the existing evidence supporting pathological loss of connectivity and selective impairment of neural circuits in ALS and FTD, two diseases which share strong genetic causes and impairment in motor and executive functions.

Original languageEnglish
Article number1208876
JournalFrontiers in Neural Circuits
Volume17
Number of pages9
ISSN1662-5110
DOIs
Publication statusPublished - 2023

Bibliographical note

Publisher Copyright:
Copyright © 2023 Mora and Allodi.

Keywords

  • amyotrophic lateral sclerosis (ALS)
  • cognitive functions
  • frontotemporal dementia (FTD)
  • motor control
  • synapses and neurons

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