Chapter 14 - Molecular Pathogenesis and Therapeutic Targets in Huntington's Disease

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• Publication year

  2006

• Venue

  Unknown venue

• Publication date

  2006-12-01

• Fields of study

  Medicine

• Identifiers
  DOI 10.1016/B978-012369462-1/50015-6
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar

• No claims are published for this paper.

• No concepts are published for this paper.

• The protective effects of cystamine in the R6/2 Huntington's disease mouse involve mechanisms other than the inhibition of tissue transglutaminase.

  2006cited by this paper
• Cerebral PET imaging and histological evidence of transglutaminase inhibitor cystamine induced neuroprotection in transgenic R6/2 mouse model of Huntington's disease.

  2005cited by this paper
• A potent small molecule inhibits polyglutamine aggregation in Huntington's disease neurons and suppresses neurodegeneration in vivo.

  2005cited by this paper
• Neuronal dysfunction in a polyglutamine disease model occurs in the absence of ubiquitin-proteasome system impairment and inversely correlates with the degree of nuclear inclusion formation.

  2005cited by this paper
• RNA interference improves motor and neuropathological abnormalities in a Huntington's disease mouse model.

  2005cited by this paper
• Lentiviral gene delivery of GDNF into the striatum of R6/2 Huntington mice fails to attenuate behavioral and neuropathological changes.

  2005cited by this paper
• A combination drug therapy improves cognition and reverses gene expression changes in a mouse model of Huntington's disease

  2005cited by this paper
• Compounds blocking mutant huntingtin toxicity identified using a Huntington's disease neuronal cell model.

  2005cited by this paper
• Polyglutamine diseases and transport problems: deadly traffic jams on neuronal highways.

  2005cited by this paper
• Identification of combinatorial drug regimens for treatment of Huntington's disease using Drosophila.

  2005cited by this paper
• Minocycline in phenotypic models of Huntington's disease.

  2005cited by this paper
• Ataxin-3 suppresses polyglutamine neurodegeneration in Drosophila by a ubiquitin-associated mechanism.

  2005cited by this paper
• Protective effects of ginseng saponins on 3-nitropropionic acid-induced striatal degeneration in rats.

  2005cited by this paper
• Global impairment of the ubiquitin-proteasome system by nuclear or cytoplasmic protein aggregates precedes inclusion body formation.

  2005cited by this paper
• Dynein mutations impair autophagic clearance of aggregate-prone proteins

  2005cited by this paper
• Arvanil, a hybrid endocannabinoid and vanilloid compound, behaves as an antihyperkinetic agent in a rat model of Huntington's disease.

  2005cited by this paper
• Proteasome function is inhibited by polyglutamine-expanded ataxin-1, the SCA1 gene product.

  2005cited by this paper
• Neuroprotective Effects of Phenylbutyrate in the N171-82Q Transgenic Mouse Model of Huntington's Disease*

  2005cited by this paper
• CGS21680 attenuates symptoms of Huntington's disease in a transgenic mouse model

  2005cited by this paper
• Reversal of a full-length mutant huntingtin neuronal cell phenotype by chemical inhibitors of polyglutamine-mediated aggregation

  2005cited by this paper
• Resveratrol rescues mutant polyglutamine cytotoxicity in nematode and mammalian neurons

  2005cited by this paper
• Gabapentin-lactam, but not gabapentin, reduces protein aggregates and improves motor performance in a transgenic mouse model of Huntington’s disease

  2004cited by this paper
• The metabotropic glutamate receptor 5 antagonist MPEP and the mGluR2 agonist LY379268 modify disease progression in a transgenic mouse model of Huntington's disease.

  2004cited by this paper
• Celastrols as Inducers of the Heat Shock Response and Cytoprotection*[boxs]

  2004cited by this paper
• Proteasome degrades soluble expanded polyglutamine completely and efficiently.

  2004cited by this paper
• Cellular toxicity of polyglutamine expansion proteins: mechanism of transcription factor deactivation.

  2004cited by this paper
• Deleterious effects of minocycline in animal models of Parkinson's disease and Huntington's disease

  2004cited by this paper
• Generalized brain and skin proteasome inhibition in Huntington's disease

  2004cited by this paper
• Progressive decrease in chaperone protein levels in a mouse model of Huntington's disease and induction of stress proteins as a therapeutic approach.

  2004cited by this paper
• Huntingtin controls neurotrophic support and survival of neurons by enhancing BDNF vesicular transport along microtubules.

  2004cited by this paper
• Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease

  2004cited by this paper
• Trehalose alleviates polyglutamine-mediated pathology in a mouse model of Huntington disease

  2004cited by this paper
• Eukaryotic proteasomes cannot digest polyglutamine sequences and release them during degradation of polyglutamine-containing proteins.

  2004cited by this paper
• Cytoplasmic aggregates trap polyglutamine-containing proteins and block axonal transport in a Drosophila model of Huntington's disease.

  2004cited by this paper
• Paroxetine retards disease onset and progression in Huntingtin mutant mice

  2004cited by this paper
• Genetic and environmental factors in the pathogenesis of Huntington’s disease

  2004cited by this paper
• Delayed onset of Huntington's disease in mice in an enriched environment correlates with delayed loss of cannabinoid CB1 receptors.

  2004cited by this paper
• CRE-Mediated Transcription Is Increased in Huntington's Disease Transgenic Mice

  2004cited by this paper
• Dendritic spine pathology and deficits in experience‐dependent dendritic plasticity in R6/1 Huntington's disease transgenic mice

  2004cited by this paper
• Can flies help humans treat neurodegenerative diseases?

  2004cited by this paper
• Huntingtin-protein interactions and the pathogenesis of Huntington's disease.

  2004cited by this paper
• Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death

  2004cited by this paper
• Rise and fall of minocycline in neuroprotection: need to promote publication of negative results.

  2004cited by this paper
• A cell-based screen for drugs to treat Huntington's disease.

  2004cited by this paper
• Hsp70 and Hsp40 attenuate formation of spherical and annular polyglutamine oligomers by partitioning monomer

  2004cited by this paper
• Inefficient degradation of truncated polyglutamine proteins by the proteasome

  2004cited by this paper
• Environmental, pharmacological, and genetic modulation of the HD phenotype in transgenic mice.

  2004cited by this paper
• Distinct aggregation and cell death patterns among different types of primary neurons induced by mutant huntingtin protein

  2004cited by this paper
• Inhibition of Calpain Cleavage of Huntingtin Reduces Toxicity

  2004cited by this paper
• Sodium butyrate ameliorates phenotypic expression in a transgenic mouse model of spinal and bulbar muscular atrophy.

  2004cited by this paper
• Modulating huntingtin half‐life alters polyglutamine‐dependent aggregate formation and cell toxicity

  2004cited by this paper
• Chemotherapy for the Brain: The Antitumor Antibiotic Mithramycin Prolongs Survival in a Mouse Model of Huntington's Disease

  2004cited by this paper
• Genome-wide RNA interference screen identifies previously undescribed regulators of polyglutamine aggregation.

  2004cited by this paper
• Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease.

  2003cited by this paper
• Pivotal role of oligomerization in expanded polyglutamine neurodegenerative disorders

  2003cited by this paper
• A cell-based assay for aggregation inhibitors as therapeutics of polyglutamine-repeat disease and validation in Drosophila

  2003cited by this paper
• Standardization and statistical approaches to therapeutic trials in the R6/2 mouse.

  2003cited by this paper
• Autophagy regulates the processing of amino terminal huntingtin fragments.

  2003cited by this paper
• Huntingtin forms toxic NH2-terminal fragment complexes that are promoted by the age-dependent decrease in proteasome activity

  2003cited by this paper
• Huntingtin aggregation and toxicity in Huntington's disease.

  2003cited by this paper
• Transgenic rat model of Huntington's disease.

  2003cited by this paper
• Increased survival and neuroprotective effects of BN82451 in a transgenic mouse model of Huntington's disease

  2003cited by this paper
• Neuronal Induction of the Immunoproteasome in Huntington's Disease

  2003cited by this paper
• Histone Deacetylase Inhibition by Sodium Butyrate Chemotherapy Ameliorates the Neurodegenerative Phenotype in Huntington's Disease Mice

  2003cited by this paper
• Creatine therapy provides neuroprotection after onset of clinical symptoms in Huntington's disease transgenic mice

  2003cited by this paper
• Heat Shock Protein 70 Chaperone Overexpression Ameliorates Phenotypes of the Spinal and Bulbar Muscular Atrophy Transgenic Mouse Model by Reducing Nuclear-Localized Mutant Androgen Receptor Protein

  2003cited by this paper
• Overexpression of heat shock protein 70 in R6/2 Huntington's disease mice has only modest effects on disease progression.

  2003cited by this paper
• Neurotechnique A Rapid Cellular FRET Assay of Polyglutamine Aggregation Identifies a Novel Inhibitor

  2003cited by this paper
• Disruption of axonal transport by loss of huntingtin or expression of pathogenic polyQ proteins in Drosophila.

  2003cited by this paper
• Fly models of Huntington's disease.

  2003cited by this paper
• Suberoylanilide hydroxamic acid, a histone deacetylase inhibitor, ameliorates motor deficits in a mouse model of Huntington's disease

  2003cited by this paper
• Chronic lithium chloride treatment has variable effects on motor behaviour and survival of mice transgenic for the Huntington's disease mutation.

  2003cited by this paper
• Structural and functional neuroprotection in a rat model of Huntington's disease by viral gene transfer of GDNF.

  2003cited by this paper
• Enriched environment and physical activity stimulate hippocampal but not olfactory bulb neurogenesis

  2003cited by this paper
• Huntingtin interacts with REST/NRSF to modulate the transcription of NRSE-controlled neuronal genes

  2003cited by this paper
• DNA vaccination against mutant huntingtin ameliorates the HDR6/2 diabetic phenotype.

  2003cited by this paper
• Dietary restriction normalizes glucose metabolism and BDNF levels, slows disease progression, and increases survival in huntingtin mutant mice

  2003cited by this paper
• Minocycline and doxycycline are not beneficial in a model of Huntington's disease

  2003cited by this paper
• Dramatic tissue-specific mutation length increases are an early molecular event in Huntington disease pathogenesis.

  2003cited by this paper
• Inducible PC12 cell model of Huntington's disease shows toxicity and decreased histone acetylation

  2003cited by this paper
• Are Huntington's and polyglutamine-based ataxias proteasome storage diseases?

  2003cited by this paper
• Minocycline is not beneficial in a phenotypic mouse model of Huntington's disease

  2003cited by this paper
• Mechanisms of transcriptional dysregulation in Huntington's disease

  2003cited by this paper
• Therapeutic Effects of Cystamine in a Murine Model of Huntington's Disease

  2002cited by this paper
• Heat shock protein 27 prevents cellular polyglutamine toxicity and suppresses the increase of reactive oxygen species caused by huntingtin.

  2002cited by this paper
• Prolonged survival and decreased abnormal movements in transgenic model of Huntington disease, with administration of the transglutaminase inhibitor cystamine

  2002cited by this paper
• Huntingtin toxicity in yeast model depends on polyglutamine aggregation mediated by a prion-like protein Rnq1

  2002cited by this paper
• Behavioral and Morphological Comparison of Two Nonhuman Primate Models of Huntington’s Disease

  2002cited by this paper
• Aggregated polyglutamine peptides delivered to nuclei are toxic to mammalian cells.

  2002cited by this paper
• Tauroursodeoxycholic acid, a bile acid, is neuroprotective in a transgenic animal model of Huntington's disease

  2002cited by this paper
• Aggregate-prone proteins with polyglutamine and polyalanine expansions are degraded by autophagy.

  2002cited by this paper
• Environmental enrichment slows disease progression in R6/2 Huntington's disease mice

  2002cited by this paper
• Proteases acting on mutant huntingtin generate cleaved products that differentially build up cytoplasmic and nuclear inclusions.

  2002cited by this paper
• Dysregulation of gene expression in the R6/2 model of polyglutamine disease: parallel changes in muscle and brain.

  2002cited by this paper
• Therapeutic Effects of Coenzyme Q10 and Remacemide in Transgenic Mouse Models of Huntington's Disease

  2002cited by this paper
• Protein surveillance machinery in brains with spinocerebellar ataxia type 3: Redistribution and differential recruitment of 26S proteasome subunits and chaperones to neuronal intranuclear inclusions

  2002cited by this paper
• The threshold for polyglutamine-expansion protein aggregation and cellular toxicity is dynamic and influenced by aging in Caenorhabditis elegans

  2002cited by this paper
• Riluzole prolongs survival time and alters nuclear inclusion formation in a transgenic mouse model of Huntington's disease

  2002cited by this paper
• A bivalent Huntingtin binding peptide suppresses polyglutamine aggregation and pathogenesis in Drosophila

  2002cited by this paper
• Lentiviral-Mediated Delivery of Mutant Huntingtin in the Striatum of Rats Induces a Selective Neuropathology Modulated by Polyglutamine Repeat Size, Huntingtin Expression Levels, and Protein Length

  2002cited by this paper

• Huntington disease skeletal muscle is hyperexcitable owing to chloride and potassium channel dysfunction

  2013cites this paper