Alterations of fractional anisotropy throughout cortico-basal ganglia gray matter in a macaque model of Huntington’s Disease

Weiss, Alison R.; Liguore, William A.; Brandon, Kristin; Wang, Xiaojie; Liu, Zheng; Kroenke, Christopher D.; McBride, Jodi L.

doi:10.1016/j.crneur.2023.100090

Cited by 1 publication

(1 citation statement)

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“…In fact, the Vonsattel neuropathological staging criteria recognize pathological changes to occur in numerous neuroanatomical regions at later stages of disease progression, including the broader cortical mantle, cerebellum, thalamus, and substantia nigra [ 19 , 20 , 21 ]. Loss of white matter integrity, believed to be both a cause and result of damage of gray matter neurons, is another pathological feature of HD and can be detected through multiple imaging modalities in HD individuals and small animal models [ 22 , 23 , 24 , 25 ].…”

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confidence: 99%

Brain-Derived Neurotrophic Factor Dysregulation as an Essential Pathological Feature in Huntington’s Disease: Mechanisms and Potential Therapeutics

2023

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Brain-derived neurotrophic factor (BDNF) is a major neurotrophin whose loss or interruption is well established to have numerous intersections with the pathogenesis of progressive neurological disorders. There is perhaps no greater example of disease pathogenesis resulting from the dysregulation of BDNF signaling than Huntington’s disease (HD)—an inherited neurodegenerative disorder characterized by motor, psychiatric, and cognitive impairments associated with basal ganglia dysfunction and the ultimate death of striatal projection neurons. Investigation of the collection of mechanisms leading to BDNF loss in HD highlights this neurotrophin’s importance to neuronal viability and calls attention to opportunities for therapeutic interventions. Using electronic database searches of existing and forthcoming research, we constructed a literature review with the overarching goal of exploring the diverse set of molecular events that trigger BDNF dysregulation within HD. We highlighted research that investigated these major mechanisms in preclinical models of HD and connected these studies to those evaluating similar endpoints in human HD subjects. We also included a special focus on the growing body of literature detailing key transcriptomic and epigenetic alterations that affect BDNF abundance in HD. Finally, we offer critical evaluation of proposed neurotrophin-directed therapies and assessed clinical trials seeking to correct BDNF expression in HD individuals.

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