Meso scale discovery-based assays for the detection of aggregated huntingtin

Machado-Joseph disease / Spinocerebellar ataxia type 3:lessons from disease pathogenesis and clues into therapy

(2018) Carlos Matos et al.   JOURNAL OF NEUROCHEMISTRY

Alzheimer's Disease and the Amyloid-β Peptide

(2017) M. Paul Murphy et al.   JOURNAL OF ALZHEIMERS DISEASE

The pathogenic exon 1 HTT protein is produced by incomplete splicing in Huntington’s disease patients

(2017) Andreas Neueder et al.   Scientific Reports

Frequency of nuclear mutant huntingtin inclusion formation in neurons and glia is cell-type-specific

(2016) Anne H.P. Jansen et al.   GLIA

Quantification of mutant huntingtin protein in cerebrospinal fluid from Huntington’s disease patients

(2015) Edward J. Wild et al.   JOURNAL OF CLINICAL INVESTIGATION

Scalable Production in Human Cells and Biochemical Characterization of Full-Length Normal and Mutant Huntingtin

(2015) Bin Huang et al.   PLoS One

Characterization of HTT Inclusion Size, Location, and Timing in the zQ175 Mouse Model of Huntington´s Disease: An In Vivo High-Content Imaging Study

(2015) Nikisha Carty et al.   PLoS One

Silencing Mutant Huntingtin by Adeno-Associated Virus-Mediated RNA Interference Ameliorates Disease Manifestations in the YAC128 Mouse Model of Huntington's Disease

(2014) Lisa M. Stanek et al.   HUMAN GENE THERAPY

Quantification Assays for Total and Polyglutamine-Expanded Huntingtin Proteins

(2014) Douglas Macdonald et al.   PLoS One

Development of an ELISA assay for the quantification of soluble huntingtin in human blood cells

(2013) Luisa Massai et al.   BMC BIOCHEMISTRY

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

Single-Stranded RNAs Use RNAi to Potently and Allele-Selectively Inhibit Mutant Huntingtin Expression

(2012) Dongbo Yu et al.   CELL

TR-FRET-Based Duplex Immunoassay Reveals an Inverse Correlation of Soluble and Aggregated Mutant huntingtin in Huntington's Disease

(2012) Barbara Baldo et al.   CHEMISTRY & BIOLOGY

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

(2012) Holly B. Kordasiewicz et al.   NEURON

Temporal Separation of Aggregation and Ubiquitination during Early Inclusion Formation in Transgenic Mice Carrying the Huntington’s Disease Mutation

(2012) Belvin Gong et al.   PLoS One

Comprehensive Behavioral and Molecular Characterization of a New Knock-In Mouse Model of Huntington’s Disease: zQ175

(2012) Liliana B. Menalled et al.   PLoS One

Synthetic zinc finger repressors reduce mutant huntingtin expression in the brain of R6/2 mice

(2012) M. Garriga-Canut et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Slow Amyloid Nucleation via α-Helix-Rich Oligomeric Intermediates in Short Polyglutamine-Containing Huntingtin Fragments

(2011) Murali Jayaraman et al.   JOURNAL OF MOLECULAR BIOLOGY

Microtiter plate quantification of mutant and wild-type huntingtin normalized to cell count

(2010) Andreas Weiss et al.   ANALYTICAL BIOCHEMISTRY

Proteolysis of Mutant Huntingtin Produces an Exon 1 Fragment That Accumulates as an Aggregated Protein in Neuronal Nuclei in Huntington Disease

(2010) Christian Landles et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Molecular Mechanisms and Potential Therapeutical Targets in Huntington's Disease

(2010) Chiara Zuccato et al.   PHYSIOLOGICAL REVIEWS

Single-step detection of mutant huntingtin in animal and human tissues: A bioassay for Huntington’s disease

(2009) Andreas Weiss et al.   ANALYTICAL BIOCHEMISTRY

Identical oligomeric and fibrillar structures captured from the brains of R6/2 and knock-in mouse models of Huntington's disease

(2009) Kirupa Sathasivam et al.   HUMAN MOLECULAR GENETICS

Systematic behavioral evaluation of Huntington's disease transgenic and knock-in mouse models

(2009) Liliana Menalled et al.   NEUROBIOLOGY OF DISEASE

Full-Length Human Mutant Huntingtin with a Stable Polyglutamine Repeat Can Elicit Progressive and Selective Neuropathogenesis in BACHD Mice

(2008) M. Gray et al.   JOURNAL OF NEUROSCIENCE