Mitochondrial signalling and homeostasis: from cell biology to neurological disease

JJ Collier, M Oláhová, TG McWilliams… - Trends in …, 2023 - cell.com
Efforts to understand how mitochondrial dysfunction contributes to neurodegeneration have
primarily focussed on the role of mitochondria in neuronal energy metabolism. However …

Tests to assess motor phenotype in mice: a user's guide

SP Brooks, SB Dunnett - Nature Reviews Neuroscience, 2009 - nature.com
The characterization of mouse models of human disease is essential for understanding the
underlying pathophysiology and developing new therapeutics. Many diseases are often …

Choosing an animal model for the study of Huntington's disease

MA Pouladi, AJ Morton, MR Hayden - Nature Reviews Neuroscience, 2013 - nature.com
Since the identification of the causative gene in Huntington's disease (HD), a number of
animal models of this disorder have been developed. A frequently asked question is: which …

Mismatch Repair Genes Mlh1 and Mlh3 Modify CAG Instability in Huntington's Disease Mice: Genome-Wide and Candidate Approaches

RM Pinto, E Dragileva, A Kirby, A Lloret, E Lopez… - PLoS …, 2013 - journals.plos.org
The Huntington's disease gene (HTT) CAG repeat mutation undergoes somatic expansion
that correlates with pathogenesis. Modifiers of somatic expansion may therefore provide …

Systematic behavioral evaluation of Huntington's disease transgenic and knock-in mouse models

L Menalled, BF El-Khodor, M Patry… - Neurobiology of …, 2009 - Elsevier
Huntington's disease (HD) is one of the few neurodegenerative diseases with a known
genetic cause, knowledge that has enabled the creation of animal models using genetic …

BDNF overexpression in the forebrain rescues Huntington's disease phenotypes in YAC128 mice

Y Xie, MR Hayden, B Xu - Journal of Neuroscience, 2010 - Soc Neuroscience
Huntington's disease (HD) is caused by an expansion of the polyglutamine tract at the N
terminus of huntingtin. This mutation reduces levels of BDNF in the striatum, likely by …

Silencing mutant huntingtin by adeno-associated virus-mediated RNA interference ameliorates disease manifestations in the YAC128 mouse model of Huntington's …

LM Stanek, SP Sardi, B Mastis, AR Richards… - Human gene …, 2014 - liebertpub.com
Huntington's disease (HD) is a fatal autosomal dominant neurodegenerative disease
caused by an increase in the number of polyglutamine residues in the huntingtin (Htt) …

Extensive early motor and non-motor behavioral deficits are followed by striatal neuronal loss in knock-in Huntington's disease mice

MA Hickey, A Kosmalska, J Enayati, R Cohen, S Zeitlin… - Neuroscience, 2008 - Elsevier
Huntington's disease is a neurodegenerative disorder, caused by an elongation of CAG
repeats in the huntingtin gene. Mice with an insertion of an expanded polyglutamine repeat …

Alternative processing of human HTT mRNA with implications for Huntington's disease therapeutics

S Fienko, C Landles, K Sathasivam, SJ McAteer… - Brain, 2022 - academic.oup.com
Huntington disease is caused by a CAG repeat expansion in exon 1 of the huntingtin gene
(HTT) that is translated into a polyglutamine stretch in the huntingtin protein (HTT). We …

[HTML][HTML] Mutant huntingtin, abnormal mitochondrial dynamics, defective axonal transport of mitochondria, and selective synaptic degeneration in Huntington's disease

PH Reddy, UP Shirendeb - Biochimica et Biophysica Acta (BBA)-Molecular …, 2012 - Elsevier
Huntington's disease (HD) is a progressive, fatal neurodegenerative disease caused by
expanded polyglutamine repeats in the HD gene. HD is characterized by chorea, seizures …