Transgenic Animal Models of Huntington's Disease.

No abstract is available for this paper.

• Publication year

  2011

• Venue

  Current topics in behavioral neurosciences

• Publication date

  2011-12-01

• Fields of study

  Biology, Medicine

• Identifiers
  DOI 10.1007/7854_2010_105PMID 21225414
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

• Mouse mutagenesis and disease models for neuropsychiatric disorders.

  2011cited by this paper
• Mutant Huntingtin in Glial Cells Exacerbates Neurological Symptoms of Huntington Disease Mice*

  2010cited by this paper
• Neuroprotective effects of L-carnitine in a transgenic animal model of Huntington's disease.

  2010cited by this paper
• Polyglutamine toxicity in non-neuronal cells

  2010cited by this paper
• Adipose tissue dysfunction tracks disease progression in two Huntington's disease mouse models.

  2009cited by this paper
• Expression of mutant huntingtin in mouse brain astrocytes causes age-dependent neurological symptoms

  2009cited by this paper
• Accumulation of ubiquitin conjugates in a polyglutamine disease model occurs without global ubiquitin/proteasome system impairment

  2009cited by this paper
• The A2A adenosine receptor rescues the urea cycle deficiency of Huntington's disease by enhancing the activity of the ubiquitin-proteasome system.

  2009cited by this paper
• Beyond the Rat Models of Human Neurodegenerative Disorders

  2009cited by this paper
• Generation of transgenic non-human primates with germline transmission

  2009cited by this paper
• Production of Germline Transgenic Prairie Voles (Microtus ochrogaster) Using Lentiviral Vectors1

  2009cited by this paper
• Mitochondrial DNA damage is a hallmark of chemically induced and the R6/2 transgenic model of Huntington's disease.

  2009cited by this paper
• Valproate ameliorates the survival and the motor performance in a transgenic mouse model of Huntington's disease.

  2009cited by this paper
• Voxel-based morphometry in the R6/2 transgenic mouse reveals differences between genotypes not seen with manual 2D morphometry.

  2009cited by this paper
• Combination therapy with Coenzyme Q10 and creatine produces additive neuroprotective effects in models of Parkinson’s and Huntington’s Diseases

  2009cited by this paper
• Decreased BDNF Levels Are a Major Contributor to the Embryonic Phenotype of Huntingtin Knockdown Zebrafish

  2009cited by this paper
• Intrastriatal CERE-120 (AAV-Neurturin) protects striatal and cortical neurons and delays motor deficits in a transgenic mouse model of Huntington's disease.

  2009cited by this paper
• The Ubiquitin-Proteasome Reporter GFPu Does Not Accumulate in Neurons of the R6/2 Transgenic Mouse Model of Huntington's Disease

  2009cited by this paper
• Neuroprotective effects of probenecid in a transgenic animal model of Huntington’s disease

  2009cited by this paper
• Histone Deacetylase Inhibition Modulates Kynurenine Pathway Activation in Yeast, Microglia, and Mice Expressing a Mutant Huntingtin Fragment*

  2008cited by this paper
• Impaired Regulation of Brain Mitochondria by Extramitochondrial Ca2+ in Transgenic Huntington Disease Rats*

  2008cited by this paper
• Increased metabolism in the R6/2 mouse model of Huntington's disease.

  2008cited by this paper
• MicroRNA miR-124 regulates neurite outgrowth during neuronal differentiation.

  2008cited by this paper
• Huntington's disease protein contributes to RNA-mediated gene silencing through association with Argonaute and P bodies

  2008cited by this paper
• Sex-dependent Effect of BAG1 in Ameliorating Motor Deficits of Huntington Disease Transgenic Mice*

  2008cited by this paper
• Rab5 modulates aggregation and toxicity of mutant huntingtin through macroautophagy in cell and fly models of Huntington disease

  2008cited by this paper
• The microRNA pathway and fragile X mental retardation protein.

  2008cited by this paper
• Regulation of endosome dynamics by Rab5 and Huntingtin-HAP40 effector complex in physiological versus pathological conditions.

  2008cited by this paper
• Towards a transgenic model of Huntington’s disease in a non-human primate

  2008cited by this paper
• Novel targets for Huntington's disease in an mTOR-independent autophagy pathway.

  2008cited by this paper
• A microRNA-based gene dysregulation pathway in Huntington's disease.

  2008cited by this paper
• Accumulation of N-terminal mutant huntingtin in mouse and monkey models implicated as a pathogenic mechanism in Huntington's disease.

  2008cited by this paper
• Excitotoxic neuronal death and the pathogenesis of Huntington's disease.

  2008cited by this paper
• Huntington's disease.

  2007cited by this paper
• Mitochondrial Sensitivity and Altered Calcium Handling Underlie Enhanced NMDA-Induced Apoptosis in YAC128 Model of Huntington's Disease

  2007cited by this paper
• Chromatin crosstalk in development and disease: lessons from REST

  2007cited by this paper
• A small-molecule therapeutic lead for Huntington's disease: Preclinical pharmacology and efficacy of C2-8 in the R6/2 transgenic mouse

  2007cited by this paper
• Characterization of Huntingtin Pathologic Fragments in Human Huntington Disease, Transgenic Mice, and Cell Models

  2007cited by this paper
• Selective degeneration in YAC mouse models of Huntington disease.

  2007cited by this paper
• Micro-RNA speciation in fetal, adult and Alzheimer's disease hippocampus

  2007cited by this paper
• Cerebellar neurodegeneration in the absence of microRNAs

  2007cited by this paper
• Neuronal Intranuclear and Neuropil Inclusions for Pathological Assessment of Huntington’s Disease

  2007cited by this paper
• Histones associated with downregulated genes are hypo-acetylated in Huntington's disease models.

  2007cited by this paper
• Dopamine-dependent long term potentiation in the dorsal striatum is reduced in the R6/2 mouse model of Huntington's disease.

  2007cited by this paper
• Dysregulation of C/EBPalpha by mutant Huntingtin causes the urea cycle deficiency in Huntington's disease.

  2007cited by this paper
• Global changes to the ubiquitin system in Huntington's disease

  2007cited by this paper
• The Hdh(Q150/Q150) knock-in mouse model of HD and the R6/2 exon 1 model develop comparable and widespread molecular phenotypes.

  2007cited by this paper
• Expression of mutated huntingtin fragment in the putamen is sufficient to produce abnormal movement in non-human primates.

  2007cited by this paper
• Role of microRNA Pathway in Mental Retardation

  2007cited by this paper
• Regulation of intracellular HAP1 trafficking

  2007cited by this paper
• Comparison of lymphoblast mitochondria from normal subjects and patients with Barth syndrome using electron microscopic tomography

  2007cited by this paper
• Histone Deacetylase 6 Inhibition Compensates for the Transport Deficit in Huntington's Disease by Increasing Tubulin Acetylation

  2007cited by this paper
• Dysregulation of C/EBPα by mutant Huntingtin causes the urea cycle deficiency in Huntington's disease

  2007cited by this paper
• Lentiviral vectors: are they the future of animal transgenesis?

  2007cited by this paper
• CAG-encoded polyglutamine length polymorphism in the human genome

  2007cited by this paper
• A MicroRNA Feedback Circuit in Midbrain Dopamine Neurons

  2007cited by this paper
• Phenotypic abnormalities in the YAC128 mouse model of Huntington disease are penetrant on multiple genetic backgrounds and modulated by strain.

  2007cited by this paper
• Longitudinal Evaluation of the Hdh (CAG)150 Knock-In Murine Model of Huntington's Disease

  2007cited by this paper
• Wild-type huntingtin participates in protein trafficking between the Golgi and the extracellular space.

  2007cited by this paper
• Widespread Disruption of Repressor Element-1 Silencing Transcription Factor/Neuron-Restrictive Silencer Factor Occupancy at Its Target Genes in Huntington's Disease

  2007cited by this paper
• Huntington's Disease: A transcriptional report card from the peripheral blood: Can it measure disease progression in Huntington's disease?

  2006cited by this paper
• Striatal neuronal apoptosis is preferentially enhanced by NMDA receptor activation in YAC transgenic mouse model of Huntington disease.

  2006cited by this paper
• Multiple pathways contribute to the pathogenesis of Huntington disease

  2006cited by this paper
• Cleavage at the caspase-6 site is required for neuronal dysfunction and degeneration due to mutant huntingtin.

  2006cited by this paper
• Huntingtin–HAP40 complex is a novel Rab5 effector that regulates early endosome motility and is up-regulated in Huntington's disease

  2006cited by this paper
• Regulation of Intracellular Trafficking of Huntingtin-Associated Protein-1 Is Critical for TrkA Protein Levels and Neurite Outgrowth

  2006cited by this paper
• Reciprocal actions of REST and a microRNA promote neuronal identity

  2006cited by this paper
• Selective neuronal degeneration in Huntington's disease.

  2006cited by this paper
• Motor and cognitive improvement by deep brain stimulation in a transgenic rat model of Huntington's disease.

  2006cited by this paper
• ESET/SETDB1 gene expression and histone H3 (K9) trimethylation in Huntington's disease

  2006cited by this paper
• Deficits of glutamate transmission in the striatum of toxic and genetic models of Huntington's disease.

  2006cited by this paper
• DNA microarray analysis of striatal gene expression in symptomatic transgenic Huntington's mice (R6/2) reveals neuroinflammation and insulin associations.

  2006cited by this paper
• Dopamine release is severely compromised in the R6/2 mouse model of Huntington's disease

  2006cited by this paper
• Decreased association of the transcription factor Sp1 with genes downregulated in Huntington's disease.

  2006cited by this paper
• Lysosomal proteases are involved in generation of N-terminal huntingtin fragments.

  2006cited by this paper
• Metabolic characterization of the R6/2 transgenic mouse model of Huntington's disease by high-resolution MAS 1H NMR spectroscopy.

  2006cited by this paper
• The future of genomic profiling of neurological diseases using blood.

  2006cited by this paper
• Expression of mutant huntingtin in glial cells contributes to neuronal excitotoxicity

  2005cited by this paper
• Microarray analysis shows that some microRNAs downregulate large numbers of target mRNAs

  2005cited by this paper
• Gene expression in Huntington's disease skeletal muscle: a potential biomarker.

  2005cited by this paper
• Cognitive Dysfunction Precedes Neuropathology and Motor Abnormalities in the YAC128 Mouse Model of Huntington's Disease

  2005cited by this paper
• Regional cortical thinning in preclinical Huntington disease and its relationship to cognition

  2005cited by this paper
• p53 mediates cellular dysfunction and behavioral abnormalities in Huntington's disease.

  2005cited by this paper
• Genome-wide expression profiling of human blood reveals biomarkers for Huntington's disease.

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

  2005cited by this paper
• A cAMP-response element binding protein-induced microRNA regulates neuronal morphogenesis.

  2005cited by this paper
• Ubiquitous GFP expression in transgenic chickens using a lentiviral vector

  2005cited by this paper
• Splicing mutation associated with Rett syndrome and an experimental approach for genetic diagnosis

  2005cited by this paper
• Genetic mouse models of Huntington's and Parkinson's diseases: illuminating but imperfect.

  2004cited by this paper
• Blockade of quinolinic acid-induced neurotoxicity by pyruvate is associated with inhibition of glial activation in a model of Huntington's disease.

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

  2004cited by this paper
• Efficient generation of transgenic pigs using equine infectious anaemia virus (EIAV) derived vector

  2004cited by this paper
• Generation of Transgenic Cattle by Lentiviral Gene Transfer into Oocytes1

  2004cited by this paper
• Biochemical and genetic interaction between the fragile X mental retardation protein and the microRNA pathway

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

  2004cited by this paper
• Chemotherapeutic deletion of CTG repeats in lymphoblast cells from DM1 patients.

  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
• Magnetic resonance imaging and spectroscopy in assessing 3-nitropropionic acid-induced brain lesions: an animal model of Huntington's disease.

  2004cited by this paper
• Genome-wide analysis of repressor element 1 silencing transcription factor/neuron-restrictive silencing factor (REST/NRSF) target genes.

  2004cited by this paper
• Genome sequence of the Brown Norway rat yields insights into mammalian evolution

  2004cited by this paper

• Exploring Cordycepin as a Neuroprotective Agent in Huntington's Disease: In Vitro and In Vivo Insights

  2025cites this paper
• Insulin-like growth factor 2 reduces Huntington’s disease aggregates via AKT and NF-κB signaling in huntington’s disease

  2025cites this paper
• miR-196a provides antioxidative neuroprotection via USP15/Nrf2 regulation in Huntington's disease.

  2023cites this paper
• miR-196a enhances polymerization of neuronal microfilaments through suppressing IMP3 and upregulating IGF2 in Huntington’s disease

  2022cites this paper
• The regulatory roles of microRNAs toward pathogenesis and treatments in Huntington's disease

  2021influential citation
• Altered microRNA expression in animal models of Huntington’s disease and potential therapeutic strategies

  2021cites this paper
• Kynurenine pathway metabolites in cerebrospinal fluid and blood as potential biomarkers in Huntington's disease

  2020cites this paper
• Local transgene expression and whole‐body transgenesis to model brain diseases in nonhuman primate

  2019cites this paper
• Fibroblast growth factor 9 activates anti‐oxidative functions of Nrf2 through ERK signalling in striatal cell models of Huntington's disease

  2019cites this paper
• Fibroblast Growth Factor 9 Suppresses Striatal Cell Death Dominantly Through ERK Signaling in Huntington’s Disease

  2018cites this paper
• miR-196a Enhances Neuronal Morphology through Suppressing RANBP10 to Provide Neuroprotection in Huntington's Disease

  2017cites this paper
• Natural Products against Huntington's Disease (HD): Implications of Neurotoxic Animal Models and Transgenics in Preclinical Studies

  2017cites this paper
• miR-196 a Enhances Neuronal Morphology through Suppressing RANBP 10 to Provide Neuroprotection in Huntington ’ s Disease

  2017cites this paper
• Lentiviral transgenesis in mice via a simple method of viral concentration.

  2016cites this paper
• Movement deficits and neuronal loss in basal ganglia in TRPC1 deficient mice

  2016cites this paper
• Behavioral phenotyping of minipigs transgenic for the Huntington gene.

  2016cites this paper
• The Potential Regulatory Mechanisms of miR-196a in Huntington’s Disease through Bioinformatic Analyses

  2015cites this paper
• The Differential Profiling of Ubiquitin‐Proteasome and Autophagy Systems in Different Tissues before the Onset of Huntington's Disease Models

  2015cites this paper
• The role of melatonin in multiple sclerosis, Huntington's disease and cerebral ischemia.

  2014cites this paper
• Production of Transgenic Nonhuman Primates

  2014cites this paper
• An MRI atlas of the mouse basal ganglia

  2014cites this paper
• miR-196a ameliorates phenotypes of Huntington disease in cell, transgenic mouse, and induced pluripotent stem cell models.

  2013cites this paper
• Progress and prospects for genetic modification of nonhuman primate models in biomedical research.

  2013cites this paper
• A Transgenic Minipig Model of Huntington's Disease

  2013cites this paper
• Genetically engineered pig models for human diseases.

  2013cites this paper
• Pathogenic Cellular Phenotypes are Germline Transmissible in a Transgenic Primate Model of Huntington's Disease

  2013influential citation
• Functional interactions within striatal microcircuit in animal models of Huntington's disease.

  2012cites this paper
• Frontal-Subcortical Dementias

  2012cites this paper
• Significantly differential diffusion of neuropathological aggregates in the brain of transgenic mice carrying N-terminal mutant huntingtin fused with green fluorescent protein

  2012cites this paper
• Molecular genetic models related to schizophrenia and psychotic illness: heuristics and challenges.

  2011cites this paper