Neural circuit and synaptic dysfunctions in ALS-FTD pathology
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Neural circuit and synaptic dysfunctions in ALS-FTD pathology. / Mora, Santiago; Allodi, Ilary.
In: Frontiers in Neural Circuits, Vol. 17, 1208876, 2023.Research output: Contribution to journal › Review › Research › peer-review
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TY - JOUR
T1 - Neural circuit and synaptic dysfunctions in ALS-FTD pathology
AU - Mora, Santiago
AU - Allodi, Ilary
N1 - Publisher Copyright: Copyright © 2023 Mora and Allodi.
PY - 2023
Y1 - 2023
N2 - 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.
AB - 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.
KW - amyotrophic lateral sclerosis (ALS)
KW - cognitive functions
KW - frontotemporal dementia (FTD)
KW - motor control
KW - synapses and neurons
U2 - 10.3389/fncir.2023.1208876
DO - 10.3389/fncir.2023.1208876
M3 - Review
C2 - 37469832
AN - SCOPUS:85165185145
VL - 17
JO - Frontiers in Neural Circuits
JF - Frontiers in Neural Circuits
SN - 1662-5110
M1 - 1208876
ER -
ID: 360336826