|1.||Parkinson Disease (Parkinson's Disease)
|2.||Alzheimer Disease (Alzheimer's Disease)
|3.||Neurodegenerative Diseases (Neurodegenerative Disease)
|5.||Chorea (Rheumatic Chorea)
|1.||Hayden, Michael R: 72 articles (12/2015 - 02/2002)|
|2.||Rubinsztein, David C: 52 articles (05/2015 - 05/2002)|
|3.||Cattaneo, Elena: 48 articles (01/2015 - 10/2002)|
|4.||Ross, Christopher A: 47 articles (06/2015 - 05/2002)|
|5.||Bates, Gillian P: 45 articles (05/2015 - 05/2002)|
|6.||Li, Xiao-Jiang: 45 articles (03/2015 - 03/2002)|
|7.||Ferrante, Robert J: 41 articles (06/2014 - 01/2002)|
|8.||Beal, M Flint: 38 articles (09/2014 - 01/2002)|
|9.||Nukina, Nobuyuki: 36 articles (02/2015 - 04/2003)|
|10.||Leavitt, Blair R: 33 articles (05/2015 - 02/2002)|
05/01/2008 - "Using cellular and Drosophila models for Huntington's disease it was also shown that reduced ESCRT levels inhibit clearance of expanded polyglutamine aggregates and aggravate their neurotoxic effect. "
07/01/2015 - "Biology laboratory students then tested the novel potential pharmaceuticals in Huntington's disease model assays, using in vitro polyglutamine peptide aggregation and in vivo lethality studies in Drosophila. "
02/15/2015 - "Although Huntington's disease is caused by the expansion of a CAG triplet repeat within the context of the 3144-amino acid huntingtin protein (HTT), studies reveal that N-terminal fragments of HTT containing the expanded PolyQ region can be produced by proteolytic processing and/or aberrant splicing. "
01/01/2012 - "Modeling the polyglutamine aggregation pathway in Huntington's disease: from basic studies to clinical applications."
02/01/2010 - "During the past few years, gene expression studies have shown that the perturbation of transcription frequently results in neuronal dysfunction in polyglutamine (PolyQ) diseases such as Huntington's disease (HD). "
11/01/2000 - "Intrastriatal injection of quinolinate has been proven to be a very useful animal model to study the pathogenesis and treatment of Huntington's disease. "
12/01/2003 - "Deficits induced by quinolinic acid lesion to the striatum in a position discrimination and reversal task are ameliorated by permanent and temporary lesion to the globus pallidus: a potential novel treatment in a rat model of Huntington's disease."
03/16/1998 - "Electrolytic lesion of globus pallidus ameliorates the behavioral and neurodegenerative effects of quinolinic acid lesion of the striatum: a potential novel treatment in a rat model of Huntington's disease."
06/01/2015 - "In the present study, the effect of treadmill exercise on spatial-learning ability and motor coordination focusing on the apoptosis in the hippocampus was investigated using quinolinic acid-induced Huntington's disease rats. "
02/15/2013 - "The present study was designed to examine the correlations between behavioural and oxidative parameters in a quinolinic acid model of Huntington's disease in rats. "
|3.||Minocycline (Cyclops)FDA LinkGeneric
12/01/2003 - "Minocycline is not beneficial in a phenotypic mouse model of Huntington's disease."
12/01/2003 - "Why minocycline is helpful in Huntington's disease."
12/01/2002 - "Maintained improvement with minocycline of a patient with advanced Huntington's disease."
10/15/2010 - "This study assessed the futility of proceeding with a Phase 3 clinical trial of minocycline as a disease-modifying treatment for Huntington's disease (HD). "
10/15/2010 - "A futility study of minocycline in Huntington's disease."
03/01/2015 - "In the present study, we investigated whether Ppt could protect against 3-nitropropionic acid (3-NP)-induced oxidative stress in a rat model of Huntington's disease (HD) and explored the mechanisms of action. "
03/01/2015 - "In this study, we examined the expression levels of FL-PGC-1α and N-truncated PGC-1α (NT-PGC-1α), a shorter but functionally active splice variant of PGC-1α protein, in N171-82Q transgenic and 3-nitropropionic acid-induced murine model of Huntington's disease (HD). "
04/01/2013 - "This study was designed to evaluate the effects of bis selenide on Huntington disease (HD)-like signs induced by 3-nitropropionic acid (3-NP) in rats. "
01/01/2013 - "This study shows that TMS can modulate the Nrf2 transcriptor factor in a Huntington's disease-like rat model induced by 3-nitropropionic acid (3-NP). "
05/03/2012 - "This study was undertaken to investigate the neuroprotective effects of ELFEF in a rat model of 3-nitropropionic acid (3NP)-induced Huntington's disease. "
|5.||Proteins (Proteins, Gene)IBA
04/01/2010 - "Cleavage and clearance of mutant Htt, and the interactions between mutant Htt and other cellular proteins are important biochemical events leading to Huntington's disease. "
07/06/2009 - "This study describes an assay for the detection of the intracellular mutant huntingtin, the causative agent of Huntington's disease, with a method that may be generally applicable to other cellular proteins. "
03/01/2004 - "Recent studies have identified several huntingtin-interacting proteins that might be associated with the normal function of huntingtin and/or involved in the pathology of Huntington's disease. "
09/01/2015 - "Huntington disease iPSCs show early molecular changes in intracellular signaling, the expression of oxidative stress proteins and the p53 pathway."
01/01/2015 - "In addition, we show that the activation of autophagy by pharmacological inhibition of GPCR reduces the accumulation of misfolded proteins and protects against behavior dysfunction in a mouse model of Huntington's disease. "
10/01/2010 - "Lack of efficacy of NMDA receptor-NR2B selective antagonists in the R6/2 model of Huntington disease."
01/01/2011 - "The aim of this study was to replicate association of variations in the N-methyl D-aspartate receptor subtype genes GRIN2A and GRIN2B in the "REGISTRY" cohort from the European Huntington Disease Network (EHDN). "
01/01/2011 - "NMDA receptor gene variations as modifiers in Huntington disease: a replication study."
07/01/1993 - "A role for the N-methyl-D-aspartate (NMDA) receptor in the molecular pathology underlying Huntington disease (HD) has been proposed on the basis of neurochemical studies in HD and the ability of the NMDA receptor to mediate neuronal cell death. "
02/01/1992 - "Recent studies in experimental animals showed that mitochondrial toxins can result in a pattern of neuronal degeneration closely resembling that seen in Huntington's disease, which can be blocked with NMDA antagonists. "
|7.||Genetic Markers (Genetic Marker)IBA
08/26/1989 - "Availability of new DNA markers, more tightly linked to the Huntington's disease (HD) locus than the original G8 (D4S10) probes, has improved predictive accuracy for both presymptomatic and prenatal exclusion testing. "
07/01/1987 - "These observations may prove helpful in the search for better genetic markers for Huntington's chorea, which maps close to D4S10."
01/01/2009 - "HD, the Huntington's disease gene, was the first autosomal defect mapped using only DNA markers, a finding in 1983 that helped to spur similar studies in many other disorders and contributed to the concept of the human genome project. "
11/01/1988 - "Improved predictive testing for Huntington disease by using three linked DNA markers."
06/01/1993 - "A linkage study with DNA markers (D4S95, D4S115, and D4S111) in Japanese Huntington disease families."
|8.||Brain-Derived Neurotrophic Factor (BDNF)IBA
06/04/2007 - "It was the aim of this study to establish triglyceride matrices as potential carriers for long-term release of brain-derived neurotrophic factor (BDNF), a potential therapeutic for Huntington's disease. "
04/01/2008 - "Brain-derived neurotrophic factor over-expression in the forebrain ameliorates Huntington's disease phenotypes in mice."
01/01/2010 - "Studies have implicated reduced levels of brain-derived neurotrophic factor (BDNF) in the pathogenesis of Huntington's disease. "
10/01/2015 - "Selective reduction of striatal mature BDNF without induction of proBDNF in the zQ175 mouse model of Huntington's disease."
01/01/2015 - "Several cellular mechanisms have been proposed to explain the pathogenesis of Huntington's disease (HD), including the lack of striatal brain-derived neurotrophic factor (BDNF). "
|9.||ethyl eicosapentaenoic acidIBA
12/01/2008 - "Ethyl-EPA was not beneficial in patients with Huntington disease during 6 months of placebo-controlled evaluation. "
01/21/2002 - "After 6 months all the patients treated with ethyl-EPA improved on the orofacial component of the Unified Huntington's Disease Rating Scale while all the patients on placebo deteriorated on this scale (p < 0.03). "
01/21/2002 - "MRI and neuropsychological improvement in Huntington disease following ethyl-EPA treatment."
12/01/2008 - "Randomized controlled trial of ethyl-eicosapentaenoic acid in Huntington disease: the TREND-HD study."
09/01/2008 - "High-resolution cerebral magnetic resonance imaging scans were acquired at baseline, 6 months and 1 year in up to 34 patients with stage I or II Huntington's disease who took part in a randomized, double-blind, placebo-controlled trial of ethyl-EPA. "
12/01/2009 - "Delayed onset of the diurnal melatonin rise in patients with Huntington's disease."
10/01/2014 - "We analyzed the circadian rhythm of melatonin in a 24-hour study of cohorts of control, premanifest, and stage II/III Huntington's disease subjects. "
10/01/2014 - "Altered melatonin patterns may provide an explanation for disrupted sleep and circadian behavior in Huntington's disease, and represent a biomarker for disease state. "
10/01/2014 - "Melatonin concentrations are reduced in Huntington's disease. "
10/01/2014 - "Onset of melatonin rise was significantly more temporally spread in both premanifest and stage II/III Huntington's disease subjects compared with controls. "
|1.||Transplantation (Transplant Recipients)
01/01/1998 - "Fetal striatal transplantation may be as feasible, safe, and effective a treatment for Huntington's disease (HD), a disorder for which there is currently no effective treatment. "
12/01/2014 - "We analyze the safety and efficacy of the intrastriatal transplantation of human fetal neuroblasts from ganglionic eminences in patients with Huntington's disease. "
06/01/2013 - "The long-term safety and efficacy of bilateral transplantation of human fetal striatal tissue in patients with mild to moderate Huntington's disease."
04/01/2006 - "Neuronal transplantation in Huntington's disease provides a period of several years of improvement and stability, but not a permanent cure for the disease. "
03/01/1996 - "Transplantation of human fetal striatum into a rodent model of Huntington's disease ameliorates locomotor deficits."
04/01/2006 - "Although we have shown in three out of five patients with Huntington's disease that motor and cognitive improvements 2 years after intracerebral fetal neural grafts are correlated with recovery of brain metabolic activity in grafted striatal areas and connected regions of the cerebral cortex, neural grafts are not known to have protective effects on the host brain per se. "
12/01/2013 - "Microtransplantation of whole ganglionic eminence cells ameliorates motor deficit, enlarges the volume of grafts, and prolongs survival in a rat model of Huntington's disease."
02/01/2013 - "In the present study, we analysed the vasculature of the host putamen and solid grafts of foetal striatal tissue transplanted into patients with Huntington's disease 9 and 12 years previously. "
04/01/2009 - "To date most studies have focused on rodent and primate models of Huntington's disease (HD) with demonstrations that transplants of CP can prevent the behavioral and anatomical consequences of striatal degeneration. "
04/01/2009 - "Potential of choroid plexus epithelial cell grafts for neuroprotection in Huntington's disease: what remains before considering clinical trials."
07/01/2004 - "The woman was evaluated with the Unified Huntington's Disease Rating Scale before and after treatment. "
06/01/1997 - "We report a 56-year-old man with Huntington's disease whose chorea substantially improved after treatment with electroconvulsive therapy (ECT). "
01/08/1980 - "The author reports a case of Huntington's chorea improved after treatment with tiapride, and discusses the complex pathogenic features of this affection and the therapeutic difficulties encountered."
05/01/2011 - "In addition, the finding of a strong relationship between clinical severity and metabolic activity after treatment also suggests that pridopidine treatment targets a Huntington disease-related metabolic activity pattern."
01/01/2006 - "All patients were treated with valproic acid and scored by using the Unified Huntington's Disease Rating Scale (UHDRS) motor score before and after treatment. "
|4.||Tissue Therapy (Cell Therapy)
04/01/2015 - "The efficient generation of striatal neurons from human embryonic stem cells (hESCs) and induced pluripotent stem cells (hiPSCs) is fundamental for realising their promise in disease modelling, pharmaceutical drug screening and cell therapy for Huntington's disease. "
12/01/2013 - "Studies have demonstrated that embryonic cell therapy is a potential approach for the treatment of Huntington's disease (HD). "
01/01/2012 - "During the past decade, the exploration of alternative cellular sources, conducted in parallel to the clinical trials, has gradually put forward human pluripotent stem cells as prime candidate for Huntington's disease cell therapy. "
12/01/2014 - "Huntington's disease is currently incurable, but cell therapy is seen as a promising alternative treatment. "
09/15/2014 - "This article presents the pros and cons of taking a cell therapy approach in Huntington's disease. "
01/01/2015 - "Finally, this study suggests that the potential use of novel therapeutics aimed at modulating the Huntington's disease innate immune system should not be extended to include the adaptive immune system. "
01/01/2015 - "Our study suggests a potential novel drug target in developing autophagy-based therapeutics for the treatment of proteinopathies including Huntington's disease. "
04/01/2011 - "As therapeutics are being developed to target the underlying neuropathology of Huntington disease, interest is increasing in methodologies for conducting clinical trials in the prodromal phase. "
03/01/2005 - "With prospects improving for experimental therapeutics aimed at postponing the onset of illness in preclinical carriers of the Huntington's disease (HD) gene, we assessed agreement among experienced clinicians with respect to the motor manifestations of HD, a relevant outcome measure for preventive trials in this population. "
08/01/2003 - "Experimental therapeutics in Huntington's disease: are models useful for therapeutic trials?"