Animal models of human disease: challenges in enabling translation
P McGonigle, B Ruggeri - Biochemical pharmacology, 2014 - Elsevier
Animal models have historically played a critical role in the exploration and characterization
of disease pathophysiology, target identification, and in the in vivo evaluation of novel …
of disease pathophysiology, target identification, and in the in vivo evaluation of novel …
Animal models of CNS disorders
P McGonigle - Biochemical pharmacology, 2014 - Elsevier
There is intense interest in the development and application of animal models of CNS
disorders to explore pathology and molecular mechanisms, identify potential biomarkers …
disorders to explore pathology and molecular mechanisms, identify potential biomarkers …
Neuronal targets for reducing mutant huntingtin expression to ameliorate disease in a mouse model of Huntington's disease
Huntington's disease (HD) is a fatal dominantly inherited neurodegenerative disorder
caused by a CAG repeat expansion leading to an elongated polyglutamine stretch in …
caused by a CAG repeat expansion leading to an elongated polyglutamine stretch in …
A Large Scale Huntingtin Protein Interaction Network Implicates Rho GTPase Signaling Pathways in Huntington Disease*♦
C Tourette, B Li, R Bell, S O'Hare, LS Kaltenbach… - Journal of Biological …, 2014 - ASBMB
Huntington disease (HD) is an inherited neurodegenerative disease caused by a CAG
expansion in the HTT gene. Using yeast two-hybrid methods, we identified a large set of …
expansion in the HTT gene. Using yeast two-hybrid methods, we identified a large set of …
HACE1 reduces oxidative stress and mutant Huntingtin toxicity by promoting the NRF2 response
B Rotblat, AL Southwell… - Proceedings of the …, 2014 - National Acad Sciences
Oxidative stress plays a key role in late onset diseases including cancer and
neurodegenerative diseases such as Huntington disease. Therefore, uncovering regulators …
neurodegenerative diseases such as Huntington disease. Therefore, uncovering regulators …
Dysfunction of the CNS-heart axis in mouse models of Huntington's disease
M Mielcarek, L Inuabasi, MK Bondulich, T Muller… - PLoS …, 2014 - journals.plos.org
Cardiac remodelling and contractile dysfunction occur during both acute and chronic
disease processes including the accumulation of insoluble aggregates of misfolded amyloid …
disease processes including the accumulation of insoluble aggregates of misfolded amyloid …
A common gene expression signature in Huntington's disease patient brain regions
Background Gene expression data provide invaluable insights into disease mechanisms. In
Huntington's disease (HD), a neurodegenerative disease caused by a tri-nucleotide repeat …
Huntington's disease (HD), a neurodegenerative disease caused by a tri-nucleotide repeat …
Differential loss of thalamostriatal and corticostriatal input to striatal projection neuron types prior to overt motor symptoms in the Q140 knock-in mouse model of …
YP Deng, T Wong, JY Wan, A Reiner - Frontiers in Systems …, 2014 - frontiersin.org
Motor slowing and forebrain white matter loss have been reported in premanifest
Huntington's disease (HD) prior to substantial striatal neuron loss. These findings raise the …
Huntington's disease (HD) prior to substantial striatal neuron loss. These findings raise the …
Possible involvement of self-defense mechanisms in the preferential vulnerability of the striatum in Huntington's disease
L Francelle, L Galvan, E Brouillet - Frontiers in cellular neuroscience, 2014 - frontiersin.org
HD is caused by a mutation in the huntingtin gene that consists in a CAG repeat expansion
translated into an abnormal poly-glutamine (polyQ) tract in the huntingtin (Htt) protein. The …
translated into an abnormal poly-glutamine (polyQ) tract in the huntingtin (Htt) protein. The …
MeCP2: a novel Huntingtin interactor
KN McFarland, MN Huizenga, SB Darnell… - Human Molecular …, 2014 - academic.oup.com
Transcriptional dysregulation has been proposed to play a major role in the pathology of
Huntington's disease (HD). However, the mechanisms that cause selective downregulation …
Huntington's disease (HD). However, the mechanisms that cause selective downregulation …