Energy Metabolism and Mitochondrial Superoxide Anion Production in Pre-symptomatic Striatal Neurons Derived from Human-Induced Pluripotent Stem Cells Expressing Mutant Huntingtin

Bioenergetic deficits in Huntington’s disease iPSC-derived neural cells and rescue with glycolytic metabolites

(2019) Amanda J Kedaigle et al.   HUMAN MOLECULAR GENETICS

Metabolic Reprogramming in Astrocytes Distinguishes Region-Specific Neuronal Susceptibility in Huntington Mice

(2019) Aris A. Polyzos et al.   Cell Metabolism

Striatal neurons directly converted from Huntington’s disease patient fibroblasts recapitulate age-associated disease phenotypes

(2018) Matheus B. Victor et al.   NATURE NEUROSCIENCE

XJB-5-131-mediated improvement in physiology and behaviour of the R6/2 mouse model of Huntington's disease is age- and sex- dependent

(2018) Aris A. Polyzos et al.   PLoS One

Oxidative metabolism and Ca 2+ handling in striatal mitochondria from YAC128 mice, a model of Huntington's disease

(2017) James Hamilton et al.   NEUROCHEMISTRY INTERNATIONAL

Reversal of Phenotypic Abnormalities by CRISPR/Cas9-Mediated Gene Correction in Huntington Disease Patient-Derived Induced Pluripotent Stem Cells

(2017) Xiaohong Xu et al.   Stem Cell Reports

Mitochondrial targeting of XJB-5-131 attenuates or improves pathophysiology in HdhQ150 animals with well-developed disease phenotypes

(2016) Aris Polyzos et al.   HUMAN MOLECULAR GENETICS

Mitochondria-targeted molecules MitoQ and SS31 reduce mutant huntingtin-induced mitochondrial toxicity and synaptic damage in Huntington's disease

(2016) Xiangling Yin et al.   HUMAN MOLECULAR GENETICS

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

Determining Maximum Glycolytic Capacity Using Extracellular Flux Measurements

(2016) Shona A. Mookerjee et al.   PLoS One

Development and function of human cerebral cortex neural networks from pluripotent stem cells in vitro

(2015) P. Kirwan et al.   DEVELOPMENT

Lack of Oxidative Stress in a Mouse Neural Stem Cell Model of Huntington's Disease

(2015) Irina V Perevoshchikova' et al.   FREE RADICAL BIOLOGY AND MEDICINE

Oxidative metabolism in YAC128 mouse model of Huntington's disease

(2015) James Hamilton et al.   HUMAN MOLECULAR GENETICS

Ca2+handling in isolated brain mitochondria and cultured neurons derived from the YAC128 mouse model of Huntington's disease

(2015) Jessica J. Pellman et al.   JOURNAL OF NEUROCHEMISTRY

Mutant Huntingtin and Elusive Defects in Oxidative Metabolism and Mitochondrial Calcium Handling

(2015) Nickolay Brustovetsky   MOLECULAR NEUROBIOLOGY

Human iPSC-Based Modeling of Late-Onset Disease via Progerin-Induced Aging

(2013) Justine D. Miller et al.   Cell Stem Cell

Early Deficits in Glycolysis Are Specific to Striatal Neurons from a Rat Model of Huntington Disease

(2013) Caroline Gouarné et al.   PLoS One

Characterization of forebrain neurons derived from late-onset Huntington's disease human embryonic stem cell lines

(2013) Jonathan C. Niclis et al.   Frontiers in Cellular Neuroscience

Quantitative proteomic analysis of induced pluripotent stem cells derived from a human Huntington's disease patient

(2012) Jung-Il Chae et al.   BIOCHEMICAL JOURNAL

Induced Pluripotent Stem Cells from Patients with Huntington's Disease Show CAG-Repeat-Expansion-Associated Phenotypes

(2012) The HD iPSC Consortium   Cell Stem Cell

Genetic Correction of Huntington's Disease Phenotypes in Induced Pluripotent Stem Cells

(2012) Mahru C. An et al.   Cell Stem Cell

The first reported generation of several induced pluripotent stem cell lines from homozygous and heterozygous Huntington's disease patients demonstrates mutation related enhanced lysosomal activity

(2012) Stefano Camnasio et al.   NEUROBIOLOGY OF DISEASE

Targeting of XJB-5-131 to Mitochondria Suppresses Oxidative DNA Damage and Motor Decline in a Mouse Model of Huntington’s Disease

(2012) Zhiyin Xun et al.   Cell Reports

Cytometric assessment of mitochondria using fluorescent probes

(2011) Cécile Cottet-Rousselle et al.   CYTOMETRY PART A

Abnormal mitochondrial dynamics, mitochondrial loss and mutant huntingtin oligomers in Huntington's disease: implications for selective neuronal damage

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

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

Mitochondria and Huntington's Disease Pathogenesis

(2010) Susan E. Browne   Annals of the New York Academy of Sciences

Mitochondrial fission and cristae disruption increase the response of cell models of Huntington's disease to apoptotic stimuli

(2010) Veronica Costa et al.   EMBO Molecular Medicine

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

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

Molecular Mechanisms and Potential Therapeutical Targets in Huntington's Disease

(2010) Chiara Zuccato et al.   PHYSIOLOGICAL REVIEWS

Mitochondria in Huntington's disease

(2009) Maria Damiano et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Mitochondrial structural and functional dynamics in Huntington's disease

(2009) P. Hemachandra Reddy et al.   BRAIN RESEARCH REVIEWS

Disease-Specific Induced Pluripotent Stem Cells

(2008) In-Hyun Park et al.   CELL

N-Terminal Mutant Huntingtin Associates with Mitochondria and Impairs Mitochondrial Trafficking

(2008) A. L. Orr et al.   JOURNAL OF NEUROSCIENCE

Mutant huntingtin and mitochondrial dysfunction

(2008) Ella Bossy-Wetzel et al.   TRENDS IN NEUROSCIENCES