An in vitro perspective on the molecular mechanisms underlying mutant huntingtin protein toxicity

Rapamycin prevents the mutant huntingtin-suppressed GLT-1 expression in cultured astrocytes

(2012) Lei-lei Chen et al.   ACTA PHARMACOLOGICA SINICA

Expression of mutant N-terminal huntingtin fragment (htt552-100Q) in astrocytes suppresses the secretion of BDNF

(2012) Linhui Wang et al.   BRAIN RESEARCH

Multiple phenotypes in Huntington disease mouse neural stem cells

(2012) James J. Ritch et al.   MOLECULAR AND CELLULAR NEUROSCIENCE

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

Optineurin inclusions occur in a minority of TDP-43 positive ALS and FTLD-TDP cases and are rarely observed in other neurodegenerative disorders

(2011) Tibor Hortobágyi et al.   ACTA NEUROPATHOLOGICA

The dysfunction of hepatic transcriptional factors in mice with Huntington's Disease

(2011) Ming-Chang Chiang et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Loss of huntingtin-associated protein 1 impairs insulin secretion from pancreatic β-cells

(2011) Austin Cape et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

Modeling Pathogenesis of Huntington’s Disease with Inducible Neuroprogenitor Cells

(2011) G. Dong et al.   CELLULAR AND MOLECULAR NEUROBIOLOGY

α-Synuclein modifies huntingtin aggregation in living cells

(2011) Federico Herrera et al.   FEBS LETTERS

α-Synuclein accumulates in huntingtin inclusions but forms independent filaments and its deficiency attenuates early phenotype in a mouse model of Huntington's disease

(2011) Cristina Tomás-Zapico et al.   HUMAN MOLECULAR GENETICS

α-Synuclein levels modulate Huntington's disease in mice

(2011) Silvia Corrochano et al.   HUMAN MOLECULAR GENETICS

Preferential accumulation of N-terminal mutant huntingtin in the nuclei of striatal neurons is regulated by phosphorylation

(2011) L. S. Havel et al.   HUMAN MOLECULAR GENETICS

Huntingtin affinity for partners is not changed by polyglutamine length: aggregation itself triggers aberrant interactions

(2011) Aurélien Davranche et al.   HUMAN MOLECULAR GENETICS

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

(2011) Ferdinando Squitieri et al.   JOURNAL OF NEURAL TRANSMISSION

Peroxisome-Proliferator-Activated Receptor Gamma Coactivator 1   Contributes to Dysmyelination in Experimental Models of Huntington's Disease

(2011) Z. Xiang et al.   JOURNAL OF NEUROSCIENCE

The role of immunity in Huntington's disease

(2011) D Soulet et al.   MOLECULAR PSYCHIATRY

Caspase signalling controls microglia activation and neurotoxicity

(2011) Miguel A. Burguillos et al.   NATURE

Activated microglia proliferate at neurites of mutant huntingtin-expressing neurons

(2011) Andrew D. Kraft et al.   NEUROBIOLOGY OF AGING

Induced pluripotent stem cell lines from Huntington's disease mice undergo neuronal differentiation while showing alterations in the lysosomal pathway

(2011) Valentina Castiglioni et al.   NEUROBIOLOGY OF DISEASE

Optineurin in Huntington's disease intranuclear inclusions

(2011) Claudia Schwab et al.   NEUROSCIENCE LETTERS

Neurotoxic protein oligomerisation associated with polyglutamine diseases

(2010) Sarah L. Hands et al.   ACTA NEUROPATHOLOGICA

A Two-Step Path to Inclusion Formation of Huntingtin Peptides Revealed by Number and Brightness Analysis

(2010) Giulia Ossato et al.   BIOPHYSICAL JOURNAL

Recent Stem Cell Advances: Induced Pluripotent Stem Cells for Disease Modeling and Stem Cell–Based Regeneration

(2010) Yoshinori Yoshida et al.   CIRCULATION

In vivo expression of polyglutamine-expanded huntingtin by mouse striatal astrocytes impairs glutamate transport: a correlation with Huntington's disease subjects

(2010) M. Faideau et al.   HUMAN MOLECULAR GENETICS

Tracking Mutant Huntingtin Aggregation Kinetics in Cells Reveals Three Major Populations That Include an Invariant Oligomer Pool

(2010) Maya A. Olshina et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mutant Huntingtin in Glial Cells Exacerbates Neurological Symptoms of Huntington Disease Mice

(2010) Jennifer Bradford et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Disruption of Peripheral Circadian Timekeeping in a Mouse Model of Huntington's Disease and Its Restoration by Temporally Scheduled Feeding

(2010) E. S. Maywood et al.   JOURNAL OF NEUROSCIENCE

Quantitative Relationships between Huntingtin Levels, Polyglutamine Length, Inclusion Body Formation, and Neuronal Death Provide Novel Insight into Huntington's Disease Molecular Pathogenesis

(2010) J. Miller et al.   JOURNAL OF NEUROSCIENCE

Hunting-ton for New Proteases: MMPs as the New Target?

(2010) Ashu Johri et al.   NEURON

Deletion of the Huntingtin Polyglutamine Stretch Enhances Neuronal Autophagy and Longevity in Mice

(2010) Shuqiu Zheng et al.   PLoS Genetics

Generation of reactive oxygen species by beta amyloid fibrils and oligomers involves different intra/extracellular pathways

(2009) Cenini Giovanna et al.   AMINO ACIDS

Overexpression of F0F1-ATP synthase α suppresses mutant huntingtin aggregation and toxicity in vitro

(2009) Hong-Quan Wang et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

DNA instability in replicating Huntington's disease lymphoblasts

(2009) Milena Cannella et al.   BMC Medical Genetics

Huntington's disease: The current state of research with peripheral tissues

(2009) Jenny Sassone et al.   EXPERIMENTAL NEUROLOGY

Selective neuronal requirement for huntingtin in the developing zebrafish

(2009) T. L. Henshall et al.   HUMAN MOLECULAR GENETICS

Mutant huntingtin interacts with  -tubulin and disrupts vesicular transport and insulin secretion

(2009) R. Smith et al.   HUMAN MOLECULAR GENETICS

Post-translational modifications of expanded polyglutamine proteins: impact on neurotoxicity

(2009) M. Pennuto et al.   HUMAN MOLECULAR GENETICS

Impaired PGC-1α function in muscle in Huntington's disease

(2009) Rajnish K. Chaturvedi et al.   HUMAN MOLECULAR GENETICS

Fibril Fragmentation Enhances Amyloid Cytotoxicity

(2009) Wei-Feng Xue et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Abnormal morphology of peripheral cell tissues from patients with Huntington disease

(2009) Ferdinando Squitieri et al.   JOURNAL OF NEURAL TRANSMISSION

Huntington’s Disease Does Not Appear to Increase the Risk of Diabetes Mellitus

(2009) T. W. Boesgaard et al.   JOURNAL OF NEUROENDOCRINOLOGY

Inducible mutant huntingtin expression in HN10 cells reproduces Huntington's disease-like neuronal dysfunction

(2009) Andreas Weiss et al.   Molecular Neurodegeneration

Association of corticobasal degeneration and Huntington's disease: Can Tau aggregates protect Huntingtin toxicity?

(2009) Dominique Caparros-Lefebvre et al.   MOVEMENT DISORDERS

Serines 13 and 16 Are Critical Determinants of Full-Length Human Mutant Huntingtin Induced Disease Pathogenesis in HD Mice

(2009) Xiaofeng Gu et al.   NEURON

Distinct neuroinflammatory profile in post-mortem human Huntingtonʼs disease

(2009) Aurelio Silvestroni et al.   NEUROREPORT

Huntington disease: pathogenesis, biomarkers, and approaches to experimental therapeutics

(2009) Christopher A. Ross et al.   PARKINSONISM & RELATED DISORDERS

Formation of Polyglutamine Inclusions in a Wide Range of Non-CNS Tissues in the HdhQ150 Knock-In Mouse Model of Huntington's Disease

(2009) Hilary Moffitt et al.   PLoS One

Expression of mutant huntingtin in mouse brain astrocytes causes age-dependent neurological symptoms

(2009) Jennifer Bradford et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Huntingtin as an essential integrator of intracellular vesicular trafficking

(2009) Juliane P. Caviston et al.   TRENDS IN CELL BIOLOGY

Evidence of apoptosis and mitochondrial abnormalities in peripheral blood cells of Huntington’s disease patients

(2008) Sandra Almeida et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Disease-Specific Induced Pluripotent Stem Cells

(2008) In-Hyun Park et al.   CELL

Cardiomyocyte Expression of a Polyglutamine Preamyloid Oligomer Causes Heart Failure

(2008) J. Scott Pattison et al.   CIRCULATION

An improved protocol for primary culture of cardiomyocyte from neonatal mice

(2008) P. Sreejit et al.   IN VITRO CELLULAR & DEVELOPMENTAL BIOLOGY-ANIMAL

A novel pathogenic pathway of immune activation detectable before clinical onset in Huntington's disease

(2008) Maria Björkqvist et al.   JOURNAL OF EXPERIMENTAL MEDICINE

Differential Activities of the Ubiquitin-Proteasome System in Neurons versus Glia May Account for the Preferential Accumulation of Misfolded Proteins in Neurons

(2008) S. Tydlacka et al.   JOURNAL OF NEUROSCIENCE

Expanded-Polyglutamine Huntingtin Protein Suppresses the Secretion and Production of a Chemokine (CCL5/RANTES) by Astrocytes

(2008) S.-Y. Chou et al.   JOURNAL OF NEUROSCIENCE

Inhibition of neurite outgrowth and promotion of cell death by cytoplasmic soluble mutant huntingtin stably transfected in mouse neuroblastoma cells

(2008) Cuifang Ye et al.   NEUROSCIENCE LETTERS

Time-lapse analysis of aggregate formation in an inducible PC12 cell model of Huntington's disease reveals time-dependent aggregate formation that transiently delays cell death

(2007) B. Gong et al.   BRAIN RESEARCH BULLETIN

Inducing huntingtin inclusion formation in primary neuronal cell culture andin vivo by high-capacity adenoviral vectors expressing truncated and full-length huntingtin with polyglutamine expansion

(2007) Bin Huang et al.   JOURNAL OF GENE MEDICINE