Alterations in Synaptic Function and Plasticity in Huntington Disease.

Alterations in Synaptic Function and Plasticity in Huntington Disease.

Publication date: May 16, 2019

Huntington disease (HD) is an inherited neurodegenerative disorder caused by an expansion of the CAG repeat region in the first exon of the huntingtin (Htt) gene. Neurodegeneration, which begins in the striatum and then spreads to other brain areas, is preceded by dysfunction in multiple aspects of neurotransmission across a variety of brain areas. This review will provide an overview of the neurochemical mediators and modulators of synaptic transmission that are disrupted in HD. This includes classical neurotransmitters like glutamate and GABA, modulators such as dopamine, adenosine and endocannabinoids, and molecules like brain-derived neurotrophic factor (BDNF) which affect neurotransmission in a more indirect manner. Alterations in the functioning of these signaling pathways can occur across multiple brain regions such as striatum, cortex and hippocampus, and affect transmission and plasticity at the synapses within these regions, which may ultimately change behaviour and contribute to the pathophysiology of HD. The current state of knowledge in this area has already yielded useful information about the causes of synaptic dysfunction and selective cell death. A full understanding of the mechanisms and consequences of disruptions in synaptic function and plasticity will lend insight into the development of the symptoms of HD, and potential drug targets for ameliorating them. This article is protected by copyright. All rights reserved.

Smith-Dijak, A.I., Sepers, M.D., and Raymond, L.A. Alterations in Synaptic Function and Plasticity in Huntington Disease. 06492. 2019 J Neurochem.

Concepts Keywords
Adenosine Huntingtin
BDNF Neurotransmitter
Brain Brain-derived neurotrophic factor
Cortex Huntington’s disease
Dopamine Synaptic plasticity
Endocannabinoids Neuroplasticity
Exon Neural circuits
GABA Neurology
Gene Molecular neuroscience
Glutamate Organ systems
Hippocampus Neuroscience
Huntingtin Branches of biology
Huntington Dysfunction
Neurodegeneration Neurodegenerative disorder
Neurodegenerative Disorder
Synaptic Plasticity
Synaptic Transmission


Type Source Name
disease MESH neurodegenerative disorder
disease DOID Huntington Disease
disease MESH Huntington Disease
disease MESH development
drug DRUGBANK Adenosine
drug DRUGBANK Dopamine
drug DRUGBANK gamma-Aminobutyric acid
disease MESH multiple


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