Regenerative Approaches in Huntington’s Disease: From Mechanistic Insights to Therapeutic Protocols

Chromosomal instability during neurogenesis in Huntington's disease

(2018) Albert Ruzo et al.   DEVELOPMENT

Translation of MicroRNA-Based Huntingtin-Lowering Therapies from Preclinical Studies to the Clinic

(2018) Jana Miniarikova et al.   MOLECULAR THERAPY

Huntington modeling improves with age

(2018) Virginia B. Mattis et al.   NATURE NEUROSCIENCE

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

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

Faulty neuronal determination and cell polarization are reverted by modulating HD early phenotypes

(2018) P. Conforti et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

hPSC-Derived Striatal Cells Generated Using a Scalable 3D Hydrogel Promote Recovery in a Huntington Disease Mouse Model

(2018) Maroof M. Adil et al.   Stem Cell Reports

AAV5-miHTT gene therapy demonstrates suppression of mutant huntingtin aggregation and neuronal dysfunction in a rat model of Huntington’s disease

(2017) J Miniarikova et al.   GENE THERAPY

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

(2017) Kalina Wiatr et al.   MOLECULAR NEUROBIOLOGY

Induction of functional dopamine neurons from human astrocytes in vitro and mouse astrocytes in a Parkinson's disease model

(2017) Pia Rivetti di Val Cervo et al.   NATURE BIOTECHNOLOGY

Developmental alterations in Huntington's disease neural cells and pharmacological rescue in cells and mice

(2017) Ryan G Lim et al.   NATURE NEUROSCIENCE

MicroRNAs in CSF as prodromal biomarkers for Huntington disease in the PREDICT-HD study

(2017) Eric R. Reed et al.   NEUROLOGY

Regenerative medicine in Huntington’s disease: Strengths and weaknesses of preclinical studies

(2017) A.M. Tartaglione et al.   NEUROSCIENCE AND BIOBEHAVIORAL REVIEWS

Differentiation of human telencephalic progenitor cells into MSNs by inducible expression of Gsx2 and Ebf1

(2017) Andrea Faedo et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Concise Review: The Use of Stem Cells for Understanding and Treating Huntington's Disease

(2017) Bronwen Connor   STEM CELLS

Intracellular Cholesterol Trafficking and Impact in Neurodegeneration

(2017) Fabian Arenas et al.   Frontiers in Molecular Neuroscience

Exosome Mediated Delivery of miR-124 Promotes Neurogenesis after Ischemia

(2017) Jialei Yang et al.   Molecular Therapy-Nucleic Acids

CYP46A1, the rate-limiting enzyme for cholesterol degradation, is neuroprotective in Huntington’s disease

(2016) Lydie Boussicault et al.   BRAIN

Identification and Successful Negotiation of a Metabolic Checkpoint in Direct Neuronal Reprogramming

(2016) Sergio Gascón et al.   Cell Stem Cell

A small molecule p75NTRligand normalizes signalling and reduces Huntington’s disease phenotypes in R6/2 and BACHD mice

(2016) Danielle A. Simmons et al.   HUMAN MOLECULAR GENETICS

Integrated genomics and proteomics define huntingtin CAG length–dependent networks in mice

(2016) Peter Langfelder et al.   NATURE NEUROSCIENCE

Mitochondrial dynamics and quality control in Huntington's disease

(2016) Pedro Guedes-Dias et al.   NEUROBIOLOGY OF DISEASE

VCP recruitment to mitochondria causes mitophagy impairment and neurodegeneration in models of Huntington’s disease

(2016) Xing Guo et al.   Nature Communications

Directly Reprogrammed Human Neurons Retain Aging-Associated Transcriptomic Signatures and Reveal Age-Related Nucleocytoplasmic Defects

(2015) Jerome Mertens et al.   Cell Stem Cell

Striatal astrocytes produce neuroblasts in an excitotoxic model of Huntington's disease

(2015) G. Nato et al.   DEVELOPMENT

Activin A directs striatal projection neuron differentiation of human pluripotent stem cells

(2015) C. Arber et al.   DEVELOPMENT

Huntington disease iPSCs show early molecular changes in intracellular signaling, the expression of oxidative stress proteins and the p53 pathway

(2015) Wojciech J. Szlachcic et al.   Disease Models & Mechanisms

Cholesterol-loaded nanoparticles ameliorate synaptic and cognitive function in Huntington's disease mice

(2015) M. Valenza et al.   EMBO Molecular Medicine

MicroRNA-124 slows down the progression of Huntington′s disease by promoting neurogenesis in the striatum

(2015) Manho Kim et al.   Neural Regeneration Research

An updated role of microRNA-124 in central nervous system disorders: a review

(2015) Yang Sun et al.   Frontiers in Cellular Neuroscience

In Vivo Reprogramming of Striatal NG2 Glia into Functional Neurons that Integrate into Local Host Circuitry

(2015) Olof Torper et al.   Cell Reports

Reduction in Subventricular Zone-Derived Olfactory Bulb Neurogenesis in a Rat Model of Huntington’s Disease Is Accompanied by Striatal Invasion of Neuroblasts

(2015) Mahesh Kandasamy et al.   PLoS One

Neurogenesis in the Striatum of the Adult Human Brain

(2014) Aurélie Ernst et al.   CELL

Targets for future clinical trials in Huntington's disease: What's in the pipeline?

(2014) Edward J. Wild et al.   MOVEMENT DISORDERS

Generation of Human Striatal Neurons by MicroRNA-Dependent Direct Conversion of Fibroblasts

(2014) Matheus B. Victor et al.   NEURON

A latent neurogenic program in astrocytes regulated by Notch signaling in the mouse

(2014) J. P. Magnusson et al.   SCIENCE

Sox2-Mediated Conversion of NG2 Glia into Induced Neurons in the Injured Adult Cerebral Cortex

(2014) Christophe Heinrich et al.   Stem Cell Reports

In Vivo Direct Reprogramming of Reactive Glial Cells into Functional Neurons after Brain Injury and in an Alzheimer’s Disease Model

(2013) Ziyuan Guo et al.   Cell Stem Cell

Binding of the repressor complex REST-mSIN3b by small molecules restores neuronal gene transcription in Huntington's disease models

(2013) Paola Conforti et al.   JOURNAL OF NEUROCHEMISTRY

A Small Molecule TrkB Ligand Reduces Motor Impairment and Neuropathology in R6/2 and BACHD Mouse Models of Huntington's Disease

(2013) D. A. Simmons et al.   JOURNAL OF NEUROSCIENCE

In vivo reprogramming of astrocytes to neuroblasts in the adult brain

(2013) Wenze Niu et al.   NATURE CELL BIOLOGY

Generation of induced neurons via direct conversion in vivo

(2013) O. Torper et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

High throughput screening for inhibitors of REST in neural derivatives of human embryonic stem cells reveals a chemical compound that promotes expression of neuronal genes

(2013) Jérémie Charbord et al.   STEM CELLS

Defining the role of the Bcl-2 family proteins in Huntington’s disease

(2013) J Sassone et al.   Cell Death & Disease

Human Embryonic Stem Cell-Derived GABA Neurons Correct Locomotion Deficits in Quinolinic Acid-Lesioned Mice

(2012) Lixiang Ma et al.   Cell Stem Cell

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

(2012) The HD iPSC Consortium   Cell Stem Cell

Developmentally coordinated extrinsic signals drive human pluripotent stem cell differentiation toward authentic DARPP-32+ medium-sized spiny neurons

(2012) A. D. Carri et al.   DEVELOPMENT

Sustained Therapeutic Reversal of Huntington's Disease by Transient Repression of Huntingtin Synthesis

(2012) Holly B. Kordasiewicz et al.   NEURON

Neuronal Properties, In Vivo Effects, and Pathology of a Huntington's Disease Patient-Derived Induced Pluripotent Stem Cells

(2012) Iksoo Jeon et al.   STEM CELLS

PPARgamma rescue of the mitochondrial dysfunction in Huntington's disease

(2011) Ming-Chang Chiang et al.   NEUROBIOLOGY OF DISEASE

What have we learned from gene expression profiles in Huntington's disease?

(2011) Tamara Seredenina et al.   NEUROBIOLOGY OF DISEASE

New Striatal Neurons in a Mouse Model of Progressive Striatal Degeneration Are Generated in both the Subventricular Zone and the Striatal Parenchyma

(2011) Federico Luzzati et al.   PLoS One

Impaired adult olfactory bulb neurogenesis in the R6/2 mouse model of Huntington's disease

(2010) Zacharias Kohl et al.   BMC NEUROSCIENCE

Uncovering the true prevalence of Huntington's disease

(2010) Laura Spinney   LANCET NEUROLOGY

Directing Astroglia from the Cerebral Cortex into Subtype Specific Functional Neurons

(2010) Christophe Heinrich et al.   PLOS BIOLOGY

The Bifunctional microRNA miR-9/miR-9* Regulates REST and CoREST and Is Downregulated in Huntington's Disease

(2009) A. N. Packer et al.   JOURNAL OF NEUROSCIENCE

Gene Dysregulation in Huntington’s Disease: REST, MicroRNAs and Beyond

(2009) Rory Johnson et al.   NEUROMOLECULAR MEDICINE

Disease-Specific Induced Pluripotent Stem Cells

(2008) In-Hyun Park et al.   CELL