Prevention of depressive behaviour in the YAC128 mouse model of Huntington disease by mutation at residue 586 of huntingtin

MA Pouladi, RK Graham, JM Karasinska, Y Xie… - Brain, 2009 - academic.oup.com
Huntington disease is a neurodegenerative disorder caused by an expanded CAG repeat in
the Huntington disease gene. The symptomatic phase of the disease is defined by the onset …

Complement cascade functions during brain development and neurodegeneration

O Fatoba, T Itokazu, T Yamashita - The FEBS Journal, 2022 - Wiley Online Library
The complement system, an essential tightly regulated innate immune system, is a key
regulator of normal central nervous system (CNS) development and function. However …

Huntington's disease: the case for genetic modifiers

JF Gusella, ME MacDonald - Genome medicine, 2009 - Springer
For almost three decades, Huntington's disease has been a prototype for the application of
genetic strategies to human disease. HD, the Huntington's disease gene, was the first …

Plasma 24S-hydroxycholesterol and caudate MRI in pre-manifest and early Huntington's disease

V Leoni, C Mariotti, SJ Tabrizi, M Valenza, EJ Wild… - Brain, 2008 - academic.oup.com
Huntington's disease (HD) is a hereditary neurodegenerative disorder for which biological
indicators of disease progression, or disease stage, would be especially important for …

Aberrant complement system activation in neurological disorders

K Ziabska, M Ziemka-Nalecz, P Pawelec… - International Journal of …, 2021 - mdpi.com
The complement system is an assembly of proteins that collectively participate in the
functions of the healthy and diseased brain. The complement system plays an important role …

Human Huntington's disease pluripotent stem cell-derived microglia develop normally but are abnormally hyper-reactive and release elevated levels of reactive …

GC O'Regan, SH Farag, CS Casey… - Journal of …, 2021 - Springer
Background Neuroinflammation may contribute to the pathogenesis of Huntington's disease,
given evidence of activated microglia and elevated levels of inflammatory molecules in …

Weight loss in neurodegenerative disorders

NA Aziz, MA van der Marck, H Pijl… - Journal of …, 2008 - Springer
Unintended weight loss frequently complicates the course of many neurodegenerative
disorders and can contribute substantially to both morbidity and mortality. This will be …

Circulating levels of inflammatory biomarkers in Huntington's disease: A systematic review and meta-analysis

AS Khaboushan, A Moeinafshar, MH Ersi… - Journal of …, 2023 - Elsevier
Background Huntington's disease (HD) is an autosomal dominant disease caused by an
abnormally high number of CAG repeats at the huntingtin-encoding gene, HTT. This genetic …

Full-length huntingtin levels modulate body weight by influencing insulin-like growth factor 1 expression

MA Pouladi, Y Xie, NH Skotte… - Human molecular …, 2010 - academic.oup.com
Levels of full-length huntingtin (FL htt) influence organ and body weight, independent of
polyglutamine length. The growth hormone-insulin like growth factor-1 (GH-IGF-1) axis is …

Huntington's disease biomarker progression profile identified by transcriptome sequencing in peripheral blood

A Mastrokolias, Y Ariyurek, JJ Goeman… - European Journal of …, 2015 - nature.com
With several therapeutic approaches in development for Huntington's disease, there is a
need for easily accessible biomarkers to monitor disease progression and therapy …