Huntington disease: new insights into molecular pathogenesis and therapeutic opportunities

SJ Tabrizi, MD Flower, CA Ross, EJ Wild - Nature Reviews Neurology, 2020 - nature.com
Huntington disease (HD) is a neurodegenerative disease caused by CAG repeat expansion
in the huntingtin gene (HTT) and involves a complex web of pathogenic mechanisms …

Brain metabolism in health, aging, and neurodegeneration

S Camandola, MP Mattson - The EMBO journal, 2017 - embopress.org
Brain cells normally respond adaptively to bioenergetic challenges resulting from ongoing
activity in neuronal circuits, and from environmental energetic stressors such as food …

Neurodegenerative diseases–is metabolic deficiency the root cause?

VR Muddapu, SAP Dharshini… - Frontiers in …, 2020 - frontiersin.org
Neurodegenerative diseases, including Alzheimer, Parkinson, Huntington, and amyotrophic
lateral sclerosis, are a prominent class of neurological diseases currently without a cure …

[HTML][HTML] Transcriptional repression of PGC-1α by mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration

L Cui, H Jeong, F Borovecki, CN Parkhurst, N Tanese… - Cell, 2006 - cell.com
Huntington's disease (HD) is an inherited neurodegenerative disease caused by a
glutamine repeat expansion in huntingtin protein. Transcriptional deregulation and altered …

Mitochondrial dysfunction in Huntington's disease

C Carmo, L Naia, C Lopes, AC Rego - Polyglutamine disorders, 2018 - Springer
Mitochondrial dysfunction has been described as an early pathological mechanism
delineating the selective neurodegeneration that occurs in Huntington's disease (HD), a …

[HTML][HTML] Treatment of Huntington's disease

S Frank - Neurotherapeutics, 2014 - Elsevier
Huntington's disease (HD) is a dominantly inherited progressive neurological disease
characterized by chorea, an involuntary brief movement that tends to flow between body …

Mechanisms of neurodegeneration in Huntington's disease

JM Gil, AC Rego - European Journal of Neuroscience, 2008 - Wiley Online Library
Huntington's disease (HD) is caused by an expansion of cytosine–adenine–guanine (CAG)
repeats in the huntingtin gene, which leads to neuronal loss in the striatum and cortex and to …

Genetics and neuropathology of Huntington's disease

A Reiner, I Dragatsis, P Dietrich - International review of neurobiology, 2011 - Elsevier
Huntington's disease (HD) is an autosomal dominant progressive neurodegenerative
disorder that prominently affects the basal ganglia, leading to affective, cognitive, behavioral …

Microglial activation in presymptomatic Huntington's disease gene carriers

YF Tai, N Pavese, A Gerhard, SJ Tabrizi, RA Barker… - Brain, 2007 - academic.oup.com
Microglial activation may play a role in the pathogenesis of Huntington's disease (HD).
Using 11C-(R)-PK11195 (PK) positron emission tomography (PET), we investigated …

The neuropsychology of Huntington's disease

JS Snowden - Archives of Clinical Neuropsychology, 2017 - academic.oup.com
Huntington's disease is an inherited, degenerative brain disease, characterized by
involuntary movements, cognitive disorder and neuropsychiatric change. Men and women …