Experimental therapeutics in transgenic mouse models of Huntington's disease (Q35752429)

10 August 2017

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title

Experimental therapeutics in transgenic mouse models of Huntington's disease (English)

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10 August 2017

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2

Robert J Ferrante

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10 August 2017

1

M Flint Beal

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10 August 2017

language of work or name

English

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publication date

1 May 2004

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Nature Reviews Neuroscience

10 August 2017

number of pages

12

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inferred from page(s)

published in

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10 August 2017

volume

5

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10 August 2017

issue

5

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10 August 2017

page(s)

373-384

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Microtubule destabilization and nuclear entry are sequential steps leading to toxicity in Huntington's disease

10 August 2017

cites work

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Environmental enrichment slows disease progression in R6/2 Huntington's disease mice

21 January 2018

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Environmental stimulation increases survival in mice transgenic for exon 1 of the Huntington's disease gene

21 January 2018

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Delaying the onset of Huntington's in mice

21 January 2018

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Environmental enrichment rescues protein deficits in a mouse model of Huntington's disease, indicating a possible disease mechanism.

21 January 2018

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Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin

21 January 2018

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Dietary restriction normalizes glucose metabolism and BDNF levels, slows disease progression, and increases survival in huntingtin mutant mice

21 January 2018

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Huntington's disease of the endocrine pancreas: insulin deficiency and diabetes mellitus due to impaired insulin gene expression

21 January 2018

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A Huntington's disease CAG expansion at the murine Hdh locus is unstable and associated with behavioural abnormalities in mice

21 January 2018

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Long glutamine tracts cause nuclear localization of a novel form of huntingtin in medium spiny striatal neurons in HdhQ92 and HdhQ111 knock-in mice

21 January 2018

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Early phenotypes that presage late-onset neurodegenerative disease allow testing of modifiers in Hdh CAG knock-in mice

21 January 2018

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Neurological abnormalities in a knock-in mouse model of Huntington's disease

21 January 2018

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Behavioural abnormalities and selective neuronal loss in HD transgenic mice expressing mutated full-length HD cDNA.

21 January 2018

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Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease

21 January 2018

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Standardization and statistical approaches to therapeutic trials in the R6/2 mouse

21 January 2018

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Caspase 3-cleaved N-terminal fragments of wild-type and mutant huntingtin are present in normal and Huntington's disease brains, associate with membranes, and undergo calpain-dependent proteolysis.

21 January 2018

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Q-VD-OPh, a broad spectrum caspase inhibitor with potent antiapoptotic properties

21 January 2018

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Apoptosis and caspases in neurodegenerative diseases

21 January 2018

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Inhibition of caspase-1 slows disease progression in a mouse model of Huntington's disease

21 January 2018

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Nonapoptotic neurodegeneration in a transgenic mouse model of Huntington's disease

21 January 2018

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Overexpression and nuclear accumulation of glyceraldehyde-3-phosphate dehydrogenase in a transgenic mouse model of Huntington’s disease

21 January 2018

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Minocycline inhibits caspase-independent and -dependent mitochondrial cell death pathways in models of Huntington's disease

21 January 2018

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Minocycline inhibits caspase-1 and caspase-3 expression and delays mortality in a transgenic mouse model of Huntington disease

21 January 2018

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Minocycline and doxycycline are not beneficial in a model of Huntington's disease

21 January 2018

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Minocycline enhances MPTP toxicity to dopaminergic neurons

21 January 2018

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Tauroursodeoxycholic acid, a bile acid, is neuroprotective in a transgenic animal model of Huntington's disease.

21 January 2018

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Aggregated polyglutamine peptides delivered to nuclei are toxic to mammalian cells

21 January 2018

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Huntingtin spheroids and protofibrils as precursors in polyglutamine fibrilization

21 January 2018

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Pivotal role of oligomerization in expanded polyglutamine neurodegenerative disorders.

21 January 2018

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Identification of benzothiazoles as potential polyglutamine aggregation inhibitors of Huntington's disease by using an automated filter retardation assay

21 January 2018

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Geldanamycin activates a heat shock response and inhibits huntingtin aggregation in a cell culture model of Huntington's disease

21 January 2018

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Aggregate-prone proteins with polyglutamine and polyalanine expansions are degraded by autophagy

21 January 2018

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Trehalose alleviates polyglutamine-mediated pathology in a mouse model of Huntington disease

21 January 2018

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Interaction between mutant ataxin-1 and PQBP-1 affects transcription and cell death

21 January 2018

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Decreased expression of striatal signaling genes in a mouse model of Huntington's disease

21 January 2018

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Increased huntingtin protein length reduces the number of polyglutamine-induced gene expression changes in mouse models of Huntington's disease

21 January 2018

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Insoluble detergent-resistant aggregates form between pathological and nonpathological lengths of polyglutamine in mammalian cells

21 January 2018

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The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription

21 January 2018

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CREB-binding protein sequestration by expanded polyglutamine

21 January 2018

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Disruption of CREB function in brain leads to neurodegeneration

21 January 2018

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CRE-mediated transcription is increased in Huntington's disease transgenic mice.

21 January 2018

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Aberrant interactions of transcriptional repressor proteins with the Huntington's disease gene product, huntingtin

21 January 2018

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Identities of sequestered proteins in aggregates from cells with induced polyglutamine expression

21 January 2018

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Expanded polyglutamine stretches interact with TAFII130, interfering with CREB-dependent transcription

21 January 2018

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Interaction of Huntington disease protein with transcriptional activator Sp1

21 January 2018

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Sp1 and TAFII130 transcriptional activity disrupted in early Huntington's disease

21 January 2018

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Histone deacetylase inhibitors reduce polyglutamine toxicity

21 January 2018

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Histone deacetylase inhibitors prevent oxidative neuronal death independent of expanded polyglutamine repeats via an Sp1-dependent pathway

21 January 2018

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Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila

21 January 2018

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Suberoylanilide hydroxamic acid, a histone deacetylase inhibitor, ameliorates motor deficits in a mouse model of Huntington's disease

21 January 2018

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Long-term treatment with sodium phenylbutyrate in ornithine transcarbamylase-deficient patients

21 January 2018

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Long-term treatment of girls with ornithine transcarbamylase deficiency

21 January 2018

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Abnormal in vivo skeletal muscle energy metabolism in Huntington's disease and dentatorubropallidoluysian atrophy

21 January 2018

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Correction of hemophilia B in canine and murine models using recombinant adeno-associated viral vectors

21 January 2018

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Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines.

21 January 2018

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Specific progressive cAMP reduction implicates energy deficit in presymptomatic Huntington's disease knock-in mice

21 January 2018

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Sequential serum-dependent activation of CREB and NRF-1 leads to enhanced mitochondrial respiration through the induction of cytochrome c.

21 January 2018

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Creatine therapy provides neuroprotection after onset of clinical symptoms in Huntington's disease transgenic mice

21 January 2018

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Creatine increase survival and delays motor symptoms in a transgenic animal model of Huntington's disease

21 January 2018

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Dichloroacetate exerts therapeutic effects in transgenic mouse models of Huntington's disease

21 January 2018

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Transgenic mice expressing a Huntington's disease mutation are resistant to quinolinic acid-induced striatal excitotoxicity

21 January 2018

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Partial resistance to malonate-induced striatal cell death in transgenic mouse models of Huntington's disease is dependent on age and CAG repeat length

21 January 2018

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Maintenance of susceptibility to neurodegeneration following intrastriatal injections of quinolinic acid in a new transgenic mouse model of Huntington's disease

21 January 2018

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Coenzyme q10 prevents apoptosis by inhibiting mitochondrial depolarization independently of its free radical scavenging property.

21 January 2018

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Coenzyme Q10 and remacemide hydrochloride ameliorate motor deficits in a Huntington's disease transgenic mouse model

21 January 2018

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Riluzole prolongs survival time and alters nuclear inclusion formation in a transgenic mouse model of Huntington's disease

21 January 2018

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Chronic lithium chloride treatment has variable effects on motor behaviour and survival of mice transgenic for the Huntington's disease mutation

21 January 2018

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DNA damage induced by polyglutamine-expanded proteins

21 January 2018

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Mitochondrial dysfunction and free radical damage in the Huntington R6/2 transgenic mouse.

21 January 2018

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Striatal oxidative damage parallels the expression of a neurological phenotype in mice transgenic for the mutation of Huntington's disease

21 January 2018

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Increased oxidative damage to DNA in a transgenic mouse model of Huntington's disease

21 January 2018

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Lipoic acid improves survival in transgenic mouse models of Huntington's disease

21 January 2018

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Stilbazulenyl nitrone, a novel antioxidant, is highly neuroprotective in focal ischemia

21 January 2018

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Increased survival and neuroprotective effects of BN82451 in a transgenic mouse model of Huntington's disease.

21 January 2018

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Transglutaminase-catalyzed inactivation of glyceraldehyde 3-phosphate dehydrogenase and alpha-ketoglutarate dehydrogenase complex by polyglutamine domains of pathological length

21 January 2018

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Tissue transglutaminase-catalyzed formation of high-molecular-weight aggregates in vitro is favored with long polyglutamine domains: a possible mechanism contributing to CAG-triplet diseases.

21 January 2018

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Transglutaminase aggregates huntingtin into nonamyloidogenic polymers, and its enzymatic activity increases in Huntington's disease brain nuclei

21 January 2018

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N(epsilon)-(gamma-L-glutamyl)-L-lysine (GGEL) is increased in cerebrospinal fluid of patients with Huntington's disease

21 January 2018

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Cystamine inhibits caspase activity. Implications for the treatment of polyglutamine disorders

21 January 2018

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Prolonged survival and decreased abnormal movements in transgenic model of Huntington disease, with administration of the transglutaminase inhibitor cystamine

21 January 2018

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DNA vaccination against mutant huntingtin ameliorates the HDR6/2 diabetic phenotype.

21 January 2018

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Alzheimer disease's double-edged vaccine

21 January 2018

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Striatal transplantation in a transgenic mouse model of Huntington's disease

21 January 2018

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Anterior cingulate cortical transplantation in transgenic Huntington's disease mice

21 January 2018

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Striatal neural grafting improves cortical metabolism in Huntington's disease patients

21 January 2018

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Transgenic rat model of Huntington's disease

21 January 2018

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Reduced activity and protein expression of NOS in R6/2 HD transgenic mice: effects of L-NAME on symptom progression

21 January 2018

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Altered neuronal nitric oxide synthase expression contributes to disease progression in Huntington's disease transgenic mice

21 January 2018

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Effects of coenzyme Q10 in early Parkinson disease: evidence of slowing of the functional decline

21 January 2018

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A cell-based assay for aggregation inhibitors as therapeutics of polyglutamine-repeat disease and validation in Drosophila

21 January 2018

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A bivalent Huntingtin binding peptide suppresses polyglutamine aggregation and pathogenesis in Drosophila

21 January 2018

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A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes.

21 January 2018

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Disruption of Axonal Transport by Loss of Huntingtin or Expression of Pathogenic PolyQ Proteins in Drosophila

31 March 2020

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Neuropathogenic forms of huntingtin and androgen receptor inhibit fast axonal transport

31 March 2020

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Plans for HDBase—a research community website for Huntington's Disease

31 March 2020

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Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice

31 March 2020

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Mutant huntingtin causes context-dependent neurodegeneration in mice with Huntington's disease.

31 March 2020

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Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease

31 March 2020

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Huntingtin aggregate-associated axonal degeneration is an early pathological event in Huntington's disease mice.

31 March 2020

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Early motor dysfunction and striosomal distribution of huntingtin microaggregates in Huntington's disease knock-in mice

31 March 2020

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Cleavage of huntingtin by apopain, a proapoptotic cysteine protease, is modulated by the polyglutamine tract

31 March 2020

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Caspase cleavage of mutant huntingtin precedes neurodegeneration in Huntington's disease.

31 March 2020

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Calpain activation in Huntington's disease.

31 March 2020

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Calpain is a major cell death effector in selective striatal degeneration induced in vivo by 3-nitropropionate: implications for Huntington's disease.

31 March 2020

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Proteases acting on mutant huntingtin generate cleaved products that differentially build up cytoplasmic and nuclear inclusions

31 March 2020

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Caspase-8 is required for cell death induced by expanded polyglutamine repeats.

31 March 2020

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Epidermal immunization by a needle-free powder delivery technology: Immunogenicity of influenza vaccine and protection in mice

31 March 2020

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Neurodegeneration in the polyglutamine diseases: Act 1, Scene 1

31 March 2020

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Interference by huntingtin and atrophin-1 with cbp-mediated transcription leading to cellular toxicity

31 March 2020

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Histone Deacetylase Inhibition by Sodium Butyrate Chemotherapy Ameliorates the Neurodegenerative Phenotype in Huntington's Disease Mice

31 March 2020

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Evidence for impairment of energy metabolism in vivo in Huntington's disease using localized 1H NMR spectroscopy

31 March 2020

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Energy metabolism defects in Huntington's disease and effects of coenzyme Q10.

31 March 2020

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Abnormal in vivo skeletal muscle energy metabolism in Huntington's disease and dentatorubropallidoluysian atrophy

31 March 2020

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Neurochemical and histologic characterization of striatal excitotoxic lesions produced by the mitochondrial toxin 3-nitropropionic acid

31 March 2020

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Chronic mitochondrial energy impairment produces selective striatal degeneration and abnormal choreiform movements in primates

31 March 2020

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Clinical correlation of striatal 1H MRS changes in Huntington's disease

31 March 2020

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Mitochondrial defect in Huntington's disease caudate nucleus

31 March 2020

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Oxidative damage and metabolic dysfunction in Huntington's disease: selective vulnerability of the basal ganglia.

31 March 2020

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Neuroprotective effects of creatine and cyclocreatine in animal models of Huntington's disease.

31 March 2020

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Neuroprotective effects of creatine in a transgenic mouse model of Huntington's disease.

31 March 2020

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Increased sensitivity to N-methyl-D-aspartate receptor-mediated excitotoxicity in a mouse model of Huntington's disease

31 March 2020

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Relations between tocopherol depletion and coenzyme Q during lipid peroxidation in rat liver mitochondria

31 March 2020

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Coenzyme Q10 reduces the toxicity of rotenone in neuronal cultures by preserving the mitochondrial membrane potential

31 March 2020

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Coenzyme Q10 and nicotinamide block striatal lesions produced by the mitochondrial toxin malonate

31 March 2020

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Therapeutic effects of coenzyme Q10 and remacemide in transgenic mouse models of Huntington's disease.

31 March 2020

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Dosage effects of riluzole in Huntington's disease: a multicenter placebo-controlled study

31 March 2020

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Mitochondrial dysfunction and free radical damage in the Huntington R6/2 transgenic mouse

31 March 2020

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Neuroprotection from delayed postischemic administration of a metalloporphyrin catalytic antioxidant.

31 March 2020

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Early and progressive accumulation of reactive microglia in the Huntington disease brain

31 March 2020

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Transglutaminase action imitates Huntington's disease: selective polymerization of Huntingtin containing expanded polyglutamine.

31 March 2020

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Suppression of aggregate formation and apoptosis by transglutaminase inhibitors in cells expressing truncated DRPLA protein with an expanded polyglutamine stretch.

31 March 2020

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Therapeutic effects of cystamine in a murine model of Huntington's disease.

31 March 2020

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Transglutaminase cross-links in intranuclear inclusions in Huntington disease

31 March 2020

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Transgenic rat model of Huntington's disease

31 March 2020

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Additive neuroprotective effects of creatine and cyclooxygenase 2 inhibitors in a transgenic mouse model of amyotrophic lateral sclerosis

31 March 2020

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A Rapid Cellular FRET Assay of Polyglutamine Aggregation Identifies a Novel Inhibitor

31 March 2020

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Oxidative stress in Huntington's disease

31 March 2020

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inferred from DOI database lookup

7 January 2021

based on heuristic

A randomized, placebo-controlled trial of coenzyme Q10 and remacemide in Huntington's disease

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inferred from DOI database lookup

7 January 2021

based on heuristic

Identifiers

DOI

10.1038/NRN1386

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10 August 2017

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