Huntington's disease: progress toward effective disease-modifying treatments and a cure

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

Haplotype Background, Repeat Length Evolution, and Huntington's Disease

(2009) Daniel Falush   AMERICAN JOURNAL OF HUMAN GENETICS

CAG Expansion in the Huntington Disease Gene Is Associated with a Specific and Targetable Predisposing Haplogroup

(2009) Simon C. Warby et al.   AMERICAN JOURNAL OF HUMAN GENETICS

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

(2009) Andreas Weiss et al.   ANALYTICAL BIOCHEMISTRY

Sustained effects of nonallele-specificHuntingtinsilencing

(2009) Valérie Drouet et al.   ANNALS OF NEUROLOGY

Progress and Challenges in RNA Interference Therapy for Huntington Disease

(2009) Scott Q. Harper   ARCHIVES OF NEUROLOGY

Inactivation of Drosophila Huntingtin affects long-term adult functioning and the pathogenesis of a Huntington's disease model

(2009) S. Zhang et al.   Disease Models & Mechanisms

A majority of Huntington's disease patients may be treatable by individualized allele-specific RNA interference

(2009) Maria Stella Lombardi et al.   EXPERIMENTAL NEUROLOGY

Huntington's disease: Silencing a brutal killer

(2009) Edith L. Pfister et al.   EXPERIMENTAL NEUROLOGY

Adipose tissue dysfunction tracks disease progression in two Huntington's disease mouse models

(2009) Jack Phan et al.   HUMAN MOLECULAR GENETICS

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

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

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

IKK phosphorylates Huntingtin and targets it for degradation by the proteasome and lysosome

(2009) Leslie Michels Thompson et al.   JOURNAL OF CELL BIOLOGY

Intrabody Gene Therapy Ameliorates Motor, Cognitive, and Neuropathological Symptoms in Multiple Mouse Models of Huntington's Disease

(2009) A. L. Southwell et al.   JOURNAL OF NEUROSCIENCE

Beyond the brain: widespread pathology in Huntington's disease

(2009) Jorien MM van der Burg et al.   LANCET NEUROLOGY

Nonallele-specific Silencing of Mutant and Wild-type Huntingtin Demonstrates Therapeutic Efficacy in Huntington's Disease Mice

(2009) Ryan L Boudreau et al.   MOLECULAR THERAPY

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

(2009) Liliana Menalled et al.   NEUROBIOLOGY OF DISEASE

Huntington’s Disease – New Perspectives Based on Neuroendocrine Changes in Rodent Models

(2009) Åsa Petersén et al.   Neurodegenerative Diseases

In vivo evidence for the selective subcortical degeneration in Huntington's disease

(2009) Gwenaëlle Douaud et al.   NEUROIMAGE

Altered white matter microstructure in the corpus callosum in Huntington's disease: Implications for cortical “disconnection”

(2009) H. Diana Rosas et al.   NEUROIMAGE

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

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

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

Huntington's disease phenocopies are clinically and genetically heterogeneous

(2008) Edward J. Wild et al.   MOVEMENT DISORDERS

Artificial miRNAs mitigate shRNA-mediated toxicity in the brain: Implications for the therapeutic development of RNAi

(2008) J. L. McBride et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA