HACE1 is essential for astrocyte mitochondrial function and influences Huntington disease phenotypes in vivo

Time-Course Analysis of Protein and Lipid Oxidation in the Brains of Yac128 Huntington's Disease Transgenic Mice

(2016) Patricia S. Brocardo et al.   REJUVENATION RESEARCH

Human glia can both induce and rescue aspects of disease phenotype in Huntington disease

(2016) Abdellatif Benraiss et al.   Nature Communications

Differences in vulnerability of neurons and astrocytes to heme oxygenase-1 modulation: Implications for mitochondrial ferritin

(2016) Xiaojun Yu et al.   Scientific Reports

PINK1 expression increases during brain development and stem cell differentiation, and affects the development of GFAP-positive astrocytes

(2016) Insup Choi et al.   Molecular Brain

The emerging role of Nrf2 in mitochondrial function

(2015) Albena T. Dinkova-Kostova et al.   FREE RADICAL BIOLOGY AND MEDICINE

Anti-semaphorin 4D immunotherapy ameliorates neuropathology and some cognitive impairment in the YAC128 mouse model of Huntington disease

(2015) Amber L. Southwell et al.   NEUROBIOLOGY OF DISEASE

Neuronal development is promoted by weakened intrinsic antioxidant defences due to epigenetic repression of Nrf2

(2015) Karen F.S. Bell et al.   Nature Communications

The Role of Intermittent Hypoxia on the Proliferative Inhibition of Rat Cerebellar Astrocytes

(2015) Sheng-Chun Chiu et al.   PLoS One

Oxidative damage and the Nrf2-ARE pathway in neurodegenerative diseases

(2014) Li Gan et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Mechanisms of synaptic dysfunction and excitotoxicity in Huntington's disease

(2014) Marja D. Sepers et al.   DRUG DISCOVERY TODAY

Impaired Brain Energy Metabolism in the BACHD Mouse Model of Huntington's Disease: Critical Role of Astrocyte–Neuron Interactions

(2014) Lydie Boussicault et al.   JOURNAL OF CEREBRAL BLOOD FLOW AND METABOLISM

The Regulation of Autophagosome Dynamics by Huntingtin and HAP1 Is Disrupted by Expression of Mutant Huntingtin, Leading to Defective Cargo Degradation

(2014) Y. C. Wong et al.   JOURNAL OF NEUROSCIENCE

In Vivo Evaluation of Candidate Allele-specific Mutant Huntingtin Gene Silencing Antisense Oligonucleotides

(2014) Amber L Southwell et al.   MOLECULAR THERAPY

Inhibition of mitochondrial protein import by mutant huntingtin

(2014) Hiroko Yano et al.   NATURE NEUROSCIENCE

Allele-Specific Suppression of Mutant Huntingtin Using Antisense Oligonucleotides: Providing a Therapeutic Option for All Huntington Disease Patients

(2014) Niels H. Skotte et al.   PLoS One

HACE1 reduces oxidative stress and mutant Huntingtin toxicity by promoting the NRF2 response

(2014) B. Rotblat et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Pink1 deficiency attenuates astrocyte proliferation through mitochondrial dysfunction, reduced akt and increased p38 mapk activation, and downregulation of egfr

(2013) Insup Choi et al.   GLIA

A mitochondrial basis for Huntington’s disease: therapeutic prospects

(2013) J. Chakraborty et al.   MOLECULAR AND CELLULAR BIOCHEMISTRY

Glutathione peroxidase activity is neuroprotective in models of Huntington's disease

(2013) Robert P Mason et al.   NATURE GENETICS

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

(2013) Mahmoud A. Pouladi et al.   NATURE REVIEWS NEUROSCIENCE

Enhanced Ca2+-dependent glutamate release from astrocytes of the BACHD Huntington's disease mouse model

(2013) William Lee et al.   NEUROBIOLOGY OF DISEASE

Nrf2 impacts cellular bioenergetics by controlling substrate availability for mitochondrial respiration

(2013) K. M. Holmstrom et al.   Biology Open

Marked differences in neurochemistry and aggregates despite similar behavioural and neuropathological features of Huntington disease in the full-length BACHD and YAC128 mice

(2012) M. A. Pouladi et al.   HUMAN MOLECULAR GENETICS

Elevated NADPH oxidase activity contributes to oxidative stress and cell death in Huntington's disease

(2012) A. Valencia et al.   HUMAN MOLECULAR GENETICS

A fully humanized transgenic mouse model of Huntington disease

(2012) Amber L. Southwell et al.   HUMAN MOLECULAR GENETICS

PGC-1  Rescues Huntington's Disease Proteotoxicity by Preventing Oxidative Stress and Promoting TFEB Function

(2012) T. Tsunemi et al.   Science Translational Medicine

Assessing mitochondrial dysfunction in cells

(2011) Martin D. Brand et al.   BIOCHEMICAL JOURNAL

Antioxidants in Huntington's disease

(2011) Ashu Johri et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Light and electron microscopic characterization of the evolution of cellular pathology in YAC128 Huntington's disease transgenic mice

(2011) Zubeyde Bayram-Weston et al.   BRAIN RESEARCH BULLETIN

Mutant huntingtin's interaction with mitochondrial protein Drp1 impairs mitochondrial biogenesis and causes defective axonal transport and synaptic degeneration in Huntington's disease

(2011) U. P. Shirendeb et al.   HUMAN MOLECULAR GENETICS

Mutant huntingtin binds the mitochondrial fission GTPase dynamin-related protein-1 and increases its enzymatic activity

(2011) Wenjun Song et al.   NATURE MEDICINE

Characterization of a commonly used mouse model of SMA reveals increased seizure susceptibility and heightened fear response in FVB/N mice

(2011) Rocky G. Gogliotti et al.   NEUROBIOLOGY OF DISEASE

Efficacy of Fumaric Acid Esters in the R6/2 and YAC128 Models of Huntington's Disease

(2011) Gisa Ellrichmann et al.   PLoS One

Triterpenoids CDDO-ethyl amide and CDDO-trifluoroethyl amide improve the behavioral phenotype and brain pathology in a transgenic mouse model of Huntington's disease

(2010) Cliona Stack et al.   FREE RADICAL BIOLOGY AND MEDICINE

Mitochondrial loss, dysfunction and altered dynamics in Huntington's disease

(2010) J. Kim et al.   HUMAN MOLECULAR GENETICS

Impairment of PGC-1alpha expression, neuropathology and hepatic steatosis in a transgenic mouse model of Huntington's disease following chronic energy deprivation

(2010) R. K. Chaturvedi et al.   HUMAN MOLECULAR GENETICS

Phosphorylation of Huntingtin at Ser421 in YAC128 Neurons Is Associated with Protection of YAC128 Neurons from NMDA-Mediated Excitotoxicity and Is Modulated by PP1 and PP2A

(2010) M. Metzler et al.   JOURNAL OF NEUROSCIENCE

PGC-1alpha Down-Regulation Affects the Antioxidant Response in Friedreich's Ataxia

(2010) Daniele Marmolino et al.   PLoS One

Stress-Activated Cap'n'collar Transcription Factors in Aging and Human Disease

(2010) G. P. Sykiotis et al.   Science Signaling

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

(2009) M. A. Pouladi et al.   BRAIN

Mouse models of Huntington disease: variations on a theme

(2009) D. E. Ehrnhoefer et al.   Disease Models & Mechanisms

Intrabody Gene Therapy Ameliorates Motor, Cognitive, and Neuropathological Symptoms in Multiple Mouse Models of Huntington's Disease

(2009) A. L. Southwell et al.   JOURNAL OF NEUROSCIENCE

The gene coding for PGC-1α modifies age at onset in Huntington's Disease

(2009) Patrick Weydt et al.   Molecular Neurodegeneration

PGC-1alpha as modifier of onset age in Huntington disease

(2009) Elahe Taherzadeh-Fard et al.   Molecular Neurodegeneration

Proteomic and oxidative stress analysis in human brain samples of Huntington disease

(2008) M Alba Sorolla et al.   FREE RADICAL BIOLOGY AND MEDICINE