Comparison of spinocerebellar ataxia type 3 mouse models identifies early gain-of-function, cell-autonomous transcriptional changes in oligodendrocytes

Protein misfolding in neurodegenerative diseases: implications and strategies

(2017) Patrick Sweeney et al.   Translational Neurodegeneration

MR Imaging in Spinocerebellar Ataxias: A Systematic Review

(2016) A. Klaes et al.   AMERICAN JOURNAL OF NEURORADIOLOGY

Targeting CAG repeat RNAs reduces Huntington’s disease phenotype independently of huntingtin levels

(2016) Laura Rué et al.   JOURNAL OF CLINICAL INVESTIGATION

Walking the tightrope: proteostasis and neurodegenerative disease

(2016) Justin J. Yerbury et al.   JOURNAL OF NEUROCHEMISTRY

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

(2016) Peter Langfelder et al.   NATURE NEUROSCIENCE

Early white matter abnormalities, progressive brain pathology and motor deficits in a novel knock-in mouse model of Huntington's disease

(2015) Jing Jin et al.   HUMAN MOLECULAR GENETICS

A new humanized ataxin-3 knock-in mouse model combines the genetic features, pathogenesis of neurons and glia and late disease onset of SCA3/MJD

(2015) Pawel M. Switonski et al.   NEUROBIOLOGY OF DISEASE

Mutant Huntingtin Downregulates Myelin Regulatory Factor-Mediated Myelin Gene Expression and Affects Mature Oligodendrocytes

(2015) Brenda Huang et al.   NEURON

Glia in the pathogenesis of neurodegenerative diseases

(2014) Alexei Verkhratsky et al.   BIOCHEMICAL SOCIETY TRANSACTIONS

Trimmomatic: a flexible trimmer for Illumina sequence data

(2014) Anthony M. Bolger et al.   BIOINFORMATICS

The expanding role for chromatin and transcription in polyglutamine disease

(2014) Ryan D Mohan et al.   CURRENT OPINION IN GENETICS & DEVELOPMENT

Transcriptional dysregulation in Huntington's disease: a failure of adaptive transcriptional homeostasis

(2014) Amit Kumar et al.   DRUG DISCOVERY TODAY

A knockin mouse model of spinocerebellar ataxia type 3 exhibits prominent aggregate pathology and aberrant splicing of the disease gene transcript

(2014) Biswarathan Ramani et al.   HUMAN MOLECULAR GENETICS

Depletion of p62 reduces nuclear inclusions and paradoxically ameliorates disease phenotypes in Huntington's model mice

(2014) Masaru Kurosawa et al.   HUMAN MOLECULAR GENETICS

An RNA-Sequencing Transcriptome and Splicing Database of Glia, Neurons, and Vascular Cells of the Cerebral Cortex

(2014) Y. Zhang et al.   JOURNAL OF NEUROSCIENCE

Age-Dependent Decrease in Chaperone Activity Impairs MANF Expression, Leading to Purkinje Cell Degeneration in Inducible SCA17 Mice

(2014) Su Yang et al.   NEURON

Proteostasis impairment in protein-misfolding and -aggregation diseases

(2014) Mark S. Hipp et al.   TRENDS IN CELL BIOLOGY

Causal analysis approaches in Ingenuity Pathway Analysis

(2013) Andreas Krämer et al.   BIOINFORMATICS

Oligodendrocyte dysfunction in the pathogenesis of amyotrophic lateral sclerosis

(2013) Thomas Philips et al.   BRAIN

Localisation of N-acetylaspartate in oligodendrocytes/myelin

(2013) Kaja Nordengen et al.   Brain Structure & Function

Widespread neuronal damage and cognitive dysfunction in spinocerebellar ataxia type 3

(2013) Tátila Martins Lopes et al.   JOURNAL OF NEUROLOGY

Genomic Landscape of Transcriptional and Epigenetic Dysregulation in Early Onset Polyglutamine Disease

(2013) L. M. Valor et al.   JOURNAL OF NEUROSCIENCE

Schwann cell involvement in the peripheral neuropathy of spinocerebellar ataxia type 3

(2013) Noriaki Suga et al.   NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY

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

(2013) K. Sathasivam et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Clinical features, neurogenetics and neuropathology of the polyglutamine spinocerebellar ataxias type 1, 2, 3, 6 and 7

(2013) Udo Rüb et al.   PROGRESS IN NEUROBIOLOGY

Mechanisms of RNA-induced toxicity in CAG repeat disorders

(2013) R Nalavade et al.   Cell Death & Disease

TopHat2: accurate alignment of transcriptomes in the presence of insertions, deletions and gene fusions

(2013) Daehwan Kim et al.   GENOME BIOLOGY

Basal and stress-induced Hsp70 are modulated by ataxin-3

(2012) Christopher P. Reina et al.   CELL STRESS & CHAPERONES

Mouse Models of Spinocerebellar Ataxia Type 3 (Machado-Joseph Disease)

(2012) Veronica F. Colomer Gould   Neurotherapeutics

Longitudinal analysis of gene expression and behaviour in the HdhQ150 mouse model of Huntington's disease

(2011) Peter Giles et al.   BRAIN RESEARCH BULLETIN

Spinocerebellar Ataxia Type 7 Cerebellar Disease Requires the Coordinated Action of Mutant Ataxin-7 in Neurons and Glia, and Displays Non-Cell-Autonomous Bergmann Glia Degeneration

(2011) S. A. Furrer et al.   JOURNAL OF NEUROSCIENCE

Cell-autonomous and non-cell-autonomous toxicity in polyglutamine diseases

(2011) Fabio Sambataro et al.   PROGRESS IN NEUROBIOLOGY

Silencing ataxin-3 mitigates degeneration in a rat model of Machado–Joseph disease: no role for wild-type ataxin-3?

(2010) Sandro Alves et al.   HUMAN MOLECULAR GENETICS

Transcriptional changes in Huntington disease identified using genome-wide expression profiling and cross-platform analysis

(2010) K. Becanovic et al.   HUMAN MOLECULAR GENETICS

Polyglutamine-induced neurodegeneration in SCA3 is not mitigated by non-expanded ataxin-3: Conclusions from double-transgenic mouse models

(2010) Jeannette Hübener et al.   NEUROBIOLOGY OF DISEASE

Splice Isoforms of the Polyglutamine Disease Protein Ataxin-3 Exhibit Similar Enzymatic yet Different Aggregation Properties

(2010) Ginny Marie Harris et al.   PLoS One

ToppGene Suite for gene list enrichment analysis and candidate gene prioritization

(2009) J. Chen et al.   NUCLEIC ACIDS RESEARCH

Acquisition of Granule Neuron Precursor Identity Is a Critical Determinant of Progenitor Cell Competence to Form Shh-Induced Medulloblastoma

(2008) Ulrich Schüller et al.   CANCER CELL

In vivo suppression of polyglutamine neurotoxicity by C-terminus of Hsp70-interacting protein (CHIP) supports an aggregation model of pathogenesis

(2008) Aislinn J. Williams et al.   NEUROBIOLOGY OF DISEASE