4.6 Review

Huntington Disease as a Neurodevelopmental Disorder and Early Signs of the Disease in Stem Cells

Related references

Note: Only part of the references are listed.
Article Developmental Biology

Formative pluripotency: the executive phase in a developmental continuum

Austin Smith

DEVELOPMENT (2017)

Article Genetics & Heredity

The HTT CAG-Expansion Mutation Determines Age at Death but Not Disease Duration in Huntington Disease

Jae Whan Keum et al.

AMERICAN JOURNAL OF HUMAN GENETICS (2016)

Article Psychiatry

Longitudinal Psychiatric Symptoms in Prodromal Huntington's Disease: A Decade of Data

Eric A. Epping et al.

AMERICAN JOURNAL OF PSYCHIATRY (2016)

Article Biochemistry & Molecular Biology

Nuclear speckles are detention centers for transcripts containing expanded CAG repeats

Martyna O. Urbanek et al.

BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE (2016)

Article Biochemical Research Methods

RNA FISH for detecting expanded repeats in human diseases

Martyna O. Urbanek et al.

METHODS (2016)

Article Neurosciences

Manifestation of Huntington's disease pathology in human induced pluripotent stem cell-derived neurons

Evgeny D. Nekrasov et al.

MOLECULAR NEURODEGENERATION (2016)

Review Neurosciences

Mitochondrial dynamics and quality control in Huntington's disease

Pedro Guedes-Dias et al.

NEUROBIOLOGY OF DISEASE (2016)

Article Multidisciplinary Sciences

Selective expression of mutant huntingtin during development recapitulates characteristic features of Huntington's disease

Aldrin E. Molero et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2016)

Article Multidisciplinary Sciences

Ablation of huntingtin in adult neurons is nondeleterious but its depletion in young mice causes acute pancreatitis

Guohao Wang et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2016)

Article Clinical Neurology

Patterns of Co-Occurring Gray Matter Concentration Loss across the Huntington Disease Prodrome

Jennifer Ashley Ciarochi et al.

FRONTIERS IN NEUROLOGY (2016)

Review Neurosciences

Induced Pluripotent Stem Cells in Huntington's Disease Research: Progress and Opportunity

Adelaide Tousley et al.

JOURNAL OF HUNTINGTONS DISEASE (2016)

Review Neurosciences

Oxidative Stress and Huntington's Disease: The Good, The Bad, and The Ugly

Amit Kumar et al.

JOURNAL OF HUNTINGTONS DISEASE (2016)

Review Biochemistry & Molecular Biology

The impairment of cholesterol metabolism in Huntington disease

Valerio Leoni et al.

BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR AND CELL BIOLOGY OF LIPIDS (2015)

Article Biochemistry & Molecular Biology

Identification of Genetic Factors that Modify Clinical Onset of Huntington's Disease

Jong-Min Lee et al.

Review Cell & Tissue Engineering

Programming and Reprogramming Cellular Age in the Era of Induced Pluripotency

Lorenz Studer et al.

CELL STEM CELL (2015)

Article Biochemistry & Molecular Biology

Performance of the 12-item WHODAS 2.0 in prodromal Huntington disease

Ji-In Kim et al.

EUROPEAN JOURNAL OF HUMAN GENETICS (2015)

Article Biochemistry & Molecular Biology

HD iPSC-derived neural progenitors accumulate in culture and are susceptible to BDNF withdrawal due to glutamate toxicity

Virginia B. Mattis et al.

HUMAN MOLECULAR GENETICS (2015)

Article Biochemistry & Molecular Biology

Htt CAG repeat expansion confers pleiotropic gains of mutant huntingtin function in chromatin regulation

Marta Biagioli et al.

HUMAN MOLECULAR GENETICS (2015)

Article Psychology, Clinical

Everyday Cognition in Prodromal Huntington Disease

Janet K. Williams et al.

NEUROPSYCHOLOGY (2015)

Article Multidisciplinary Sciences

Discovery of Novel Isoforms of Huntingtin Reveals a New Hominid-Specific Exon

Albert Ruzo et al.

PLOS ONE (2015)

Review Neurosciences

Autophagy in Huntington disease and huntingtin in autophagy

Dale D. O. Martin et al.

TRENDS IN NEUROSCIENCES (2015)

Review Biochemistry & Molecular Biology

Pluripotent stem cell energy metabolism: an update

Tara Teslaa et al.

EMBO JOURNAL (2015)

Review Neurosciences

Abnormal Weight and Body Mass Index in Children with Juvenile Huntington's Disease

Alexander Tereshchenko et al.

JOURNAL OF HUNTINGTONS DISEASE (2015)

Review Biochemistry & Molecular Biology

Repeat instability during DNA repair: Insights from model systems

Karen Usdin et al.

CRITICAL REVIEWS IN BIOCHEMISTRY AND MOLECULAR BIOLOGY (2015)

Article Pharmacology & Pharmacy

Mitochondrial Biogenesis: A Therapeutic Target for Neurodevelopmental Disorders and Neurodegenerative Diseases

Martine Uittenbogaard et al.

CURRENT PHARMACEUTICAL DESIGN (2014)

Article Developmental Biology

Stem cells and the impact of ROS signaling

Carolina L. Bigarella et al.

DEVELOPMENT (2014)

Review Pharmacology & Pharmacy

The many faces of autophagy dysfunction in Huntington's disease: from mechanism to therapy

Constanza J. Cortes et al.

DRUG DISCOVERY TODAY (2014)

Review Biochemistry & Molecular Biology

Stem Cells: The Pursuit of Genomic Stability

Saranya P. Wyles et al.

INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES (2014)

Review Cell Biology

Genome maintenance in pluripotent stem cells

Uri Weissbein et al.

JOURNAL OF CELL BIOLOGY (2014)

Review Neurosciences

Self-Organization of Neural Tissue Architectures From Pluripotent Stem Cells

Michael Karus et al.

JOURNAL OF COMPARATIVE NEUROLOGY (2014)

Article Neurosciences

Mutant Huntingtin Affects Cortical Progenitor Cell Division and Development of the Mouse Neocortex

Maria Molina-Calavita et al.

JOURNAL OF NEUROSCIENCE (2014)

Article Neurosciences

Huntingtin Is Required for Normal Excitatory Synapse Development in Cortical and Striatal Circuits

Spencer U. McKinstry et al.

JOURNAL OF NEUROSCIENCE (2014)

Review Clinical Neurology

Targets for Future Clinical Trials in Huntington's Disease: What's in the Pipeline?

Edward J. Wild et al.

MOVEMENT DISORDERS (2014)

Article Multidisciplinary Sciences

Inappropriate p53 activation during development induces features of CHARGE syndrome

Jeanine L. Van Nostrand et al.

NATURE (2014)

Article Multidisciplinary Sciences

Abnormalities in human pluripotent cells due to reprogramming mechanisms

Hong Ma et al.

NATURE (2014)

Article Multidisciplinary Sciences

Allele-Specific Silencing of Mutant Huntingtin in Rodent Brain and Human Stem Cells

Valerie Drouet et al.

PLOS ONE (2014)

Article Geriatrics & Gerontology

Clinical and biomarker changes in premanifest Huntington disease show trial feasibility: a decade of the PREDICT-HD study

Jane S. Paulsen et al.

FRONTIERS IN AGING NEUROSCIENCE (2014)

Review Neurosciences

Kinase Signalling in Huntington's Disease

Kathryn R. Bowles et al.

JOURNAL OF HUNTINGTONS DISEASE (2014)

Review Cell Biology

Autophagy Regulation and role in development

Amber N. Hale et al.

AUTOPHAGY (2013)

Review Cell Biology

Autophagy in stem cells

Jun-Lin Guan et al.

AUTOPHAGY (2013)

Review Cell & Tissue Engineering

Genetic and Epigenetic Variations in iPSCs: Potential Causes and Implications for Application

Gaoyang Liang et al.

CELL STEM CELL (2013)

Review Immunology

The Role of the Immune System in Huntington's Disease

Gisa Ellrichmann et al.

CLINICAL & DEVELOPMENTAL IMMUNOLOGY (2013)

Article Biochemistry & Molecular Biology

The personal experience of parenting a child with Juvenile Huntington's Disease: perceptions across Europe

Virginia Eatough et al.

EUROPEAN JOURNAL OF HUMAN GENETICS (2013)

Article Medicine, Research & Experimental

Inhibition of mitochondrial fragmentation diminishes Huntington's disease-associated neurodegeneration

Xing Guo et al.

JOURNAL OF CLINICAL INVESTIGATION (2013)

Article Biochemistry & Molecular Biology

The ConsensusPathDB interaction database: 2013 update

Atanas Kamburov et al.

NUCLEIC ACIDS RESEARCH (2013)

Article Multidisciplinary Sciences

Selective Roles of Normal and Mutant Huntingtin in Neural Induction and Early Neurogenesis

Giang D. Nguyen et al.

PLOS ONE (2013)

Article Multidisciplinary Sciences

Functions of Huntingtin in Germ Layer Specification and Organogenesis

Giang D. Nguyen et al.

PLOS ONE (2013)

Article Multidisciplinary Sciences

Aberrant splicing of HTT generates the pathogenic exon 1 protein in Huntington disease

Kirupa Sathasivam et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2013)

Article Cell & Tissue Engineering

Oxidative stress causes DNA triplet expansion in Huntington's disease mouse embryonic stem cells

Ida Jonson et al.

STEM CELL RESEARCH (2013)

Article Cell & Tissue Engineering

Pathogenic Cellular Phenotypes are Germline Transmissible in a Transgenic Primate Model of Huntington's Disease

Kittiphong Putkhao et al.

STEM CELLS AND DEVELOPMENT (2013)

Article Neurosciences

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

Jonathan C. Niclis et al.

FRONTIERS IN CELLULAR NEUROSCIENCE (2013)

Review Neurosciences

Huntingtin: Here, There, Everywhere!

Cristovao Marques Sousa et al.

JOURNAL OF HUNTINGTONS DISEASE (2013)

Review Cell & Tissue Engineering

Epigenetic Alterations in Human Pluripotent Stem Cells: A Tale of Two Cultures

Anton Wutz

CELL STEM CELL (2012)

Article Cell & Tissue Engineering

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

Mahru C. An et al.

CELL STEM CELL (2012)

Review Developmental Biology

BDNF control of adult SVZ neurogenesis

Kevin G. Bath et al.

DEVELOPMENTAL PSYCHOBIOLOGY (2012)

Review Neurosciences

Protein aggregates in Huntington's disease

Montserrat Arrasate et al.

EXPERIMENTAL NEUROLOGY (2012)

Article Neurosciences

Multiple phenotypes in Huntington disease mouse neural stem cells

James J. Ritch et al.

MOLECULAR AND CELLULAR NEUROSCIENCE (2012)

Article Clinical Neurology

Measures of growth in children at risk for Huntington disease

Jessica K. Lee et al.

NEUROLOGY (2012)

Review Physiology

CADHERINS IN BRAIN MORPHOGENESIS AND WIRING

Shinji Hirano et al.

PHYSIOLOGICAL REVIEWS (2012)

Article Cell & Tissue Engineering

Developmental Origin of Neural Stem Cells: The Glial Cell That Could

Laura Grabel

STEM CELL REVIEWS AND REPORTS (2012)

Article Cell Biology

Signaling in Cell Differentiation and Morphogenesis

M. Albert Basson

COLD SPRING HARBOR PERSPECTIVES IN BIOLOGY (2012)

Article Neurosciences

Deletion of the Huntingtin Proline-Rich Region does not Significantly Affect Normal Huntingtin Function in Mice

Michelle Neveklovska et al.

JOURNAL OF HUNTINGTONS DISEASE (2012)

Article Biochemistry & Molecular Biology

HD CAG-correlated gene expression changes support a simple dominant gain of function

Jessie C. Jacobsen et al.

HUMAN MOLECULAR GENETICS (2011)

Article Biochemistry & Molecular Biology

Striatal neurons expressing full-length mutant huntingtin exhibit decreased N-cadherin and altered neuritogenesis

Surya A. Reis et al.

HUMAN MOLECULAR GENETICS (2011)

Article Biochemistry & Molecular Biology

Mitotic spindle: Focus on the function of huntingtin

Juliette D. Godin et al.

INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY (2011)

Review Medicine, Research & Experimental

Energy deficit in Huntington disease: why it matters

Fanny Mochel et al.

JOURNAL OF CLINICAL INVESTIGATION (2011)

Article Clinical Neurology

Genotype-, aging-dependent abnormal caspase activity in Huntington disease blood cells

Ferdinando Squitieri et al.

JOURNAL OF NEURAL TRANSMISSION (2011)

Article Cell & Tissue Engineering

Derivation of Huntington's Disease-Affected Human Embryonic Stem Cell Lines

Cara K. Bradley et al.

STEM CELLS AND DEVELOPMENT (2011)

Article Cell Biology

Monkey hybrid stem cells develop cellular features of Huntington's disease

Chuti Laowtammathron et al.

BMC CELL BIOLOGY (2010)

Article Cell & Tissue Engineering

Reprogramming Huntington Monkey Skin Cells into Pluripotent Stem Cells

Anthony W. S. Chan et al.

CELLULAR REPROGRAMMING (2010)

Article Biochemistry & Molecular Biology

Mutant huntingtin-impaired degradation of β-catenin causes neurotoxicity in Huntington's disease

Juliette D. Godin et al.

EMBO JOURNAL (2010)

Article Cell Biology

High-efficiency derivation of human embryonic stem cell lines following pre-implantation genetic diagnosis

Philippe Tropel et al.

IN VITRO CELLULAR & DEVELOPMENTAL BIOLOGY-ANIMAL (2010)

Review Genetics & Heredity

Mechanisms of trinucleotide repeat instability during human development

Cynthia T. McMurray

NATURE REVIEWS GENETICS (2010)

Review Neurosciences

Neural stem cell systems: physiological players or in vitro entities?

Luciano Conti et al.

NATURE REVIEWS NEUROSCIENCE (2010)

Review Physiology

Molecular Mechanisms and Potential Therapeutical Targets in Huntington's Disease

Chiara Zuccato et al.

PHYSIOLOGICAL REVIEWS (2010)

Article Neurosciences

Abnormal Motor Cortex Excitability in Preclinical and Very Early Huntington's Disease

Sven Schippling et al.

BIOLOGICAL PSYCHIATRY (2009)

Article Biochemistry & Molecular Biology

The non-apoptotic role of p53 in neuronal biology: enlightening the dark side of the moon

Andrea Tedeschi et al.

EMBO REPORTS (2009)

Article Biochemistry & Molecular Biology

Congenital hydrocephalus associated with abnormal subcommissural organ in mice lacking huntingtin in Wnt1 cell lineages

Paula Dietrich et al.

HUMAN MOLECULAR GENETICS (2009)

Article Multidisciplinary Sciences

Impairment of developmental stem cell-mediated striatal neurogenesis and pluripotency genes in a knock-in model of Huntington's disease

Aldrin E. Molero et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2009)

Article Obstetrics & Gynecology

Human embryonic stem cell models of Huntington disease

J. C. Niclis et al.

REPRODUCTIVE BIOMEDICINE ONLINE (2009)

Article Biochemistry & Molecular Biology

Disease-specific induced pluripotent stem cells

In-Hyun Park et al.

Article Clinical Neurology

Detection of Huntington's disease decades before diagnosis: the Predict-HD study

J. S. Paulsen et al.

JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY (2008)

Article Multidisciplinary Sciences

The ground state of embryonic stem cell self-renewal

Qi-Long Ying et al.

NATURE (2008)

Article Biochemistry & Molecular Biology

Triplet repeat mutation length gains correlate with cell-type specific vulnerability in Huntington disease brain

Peggy F. Shelbourne et al.

HUMAN MOLECULAR GENETICS (2007)

Article Biochemistry & Molecular Biology

Huntingtin inhibits caspase-3 activation

Yu Zhang et al.

EMBO JOURNAL (2006)

Article Biochemistry & Molecular Biology

Aberrant cortical synaptic plasticity and dopaminergic dysfunction in a mouse model of Huntington's disease

Damian M. Cummings et al.

HUMAN MOLECULAR GENETICS (2006)

Article Biochemistry & Molecular Biology

Keeping p53 in check: essential and synergistic functions of Mdm2 and Mdm4

J-C Marine et al.

CELL DEATH AND DIFFERENTIATION (2006)

Article Biochemistry & Molecular Biology

Early development of aberrant synaptic plasticity in a mouse model of Huntington's disease

AJ Milnerwood et al.

HUMAN MOLECULAR GENETICS (2006)

Article Biochemistry & Molecular Biology

Regional and cellular gene expression changes in human Huntington's disease brain

A Hodges et al.

HUMAN MOLECULAR GENETICS (2006)

Article Biochemistry & Molecular Biology

Niche-independent symmetrical self-renewal of a mammalian tissue stem cell

L Conti et al.

PLOS BIOLOGY (2005)

Article Obstetrics & Gynecology

Human embryonic stem cell lines with genetic disorders

Y Verlinsky et al.

REPRODUCTIVE BIOMEDICINE ONLINE (2005)

Article Biochemistry & Molecular Biology

Specific caspase interactions and amplification are involved in selective neuronal vulnerability in Huntington's disease

E Hermel et al.

CELL DEATH AND DIFFERENTIATION (2004)

Article Biochemistry & Molecular Biology

The HD mutation causes progressive lethal neurological disease in mice expressing reduced levels of huntingtin

W Auerbach et al.

HUMAN MOLECULAR GENETICS (2001)