| 1 |
35246273 | Hornerin deposits in neuronal intranuclear inclusion disease: direct identification of proteins with compositionally biased regions in inclusions. | 2022 Mar 4 |
1 |
| 2 |
35305696 | Siah-1-interacting protein regulates mutated huntingtin protein aggregation in Huntington's disease models. | 2022 Mar 19 |
1 |
| 3 |
32754987 | Impaired intracellular trafficking of sodium-dependent vitamin C transporter 2 contributes to the redox imbalance in Huntington's disease. | 2021 Jan |
1 |
| 4 |
33920936 | Emerging Roles of Exosomes in Huntington's Disease. | 2021 Apr 15 |
1 |
| 5 |
32341997 | Live-Cell Imaging and Quantification of PolyQ Aggregates by Stimulated Raman Scattering of Selective Deuterium Labeling. | 2020 Apr 22 |
1 |
| 6 |
30902619 | Novel proteomic changes in brain mitochondria provide insights into mitochondrial dysfunction in mouse models of Huntington's disease. | 2019 Jul |
2 |
| 7 |
29233555 | Human Neural Stem Cell Transplantation Rescues Functional Deficits in R6/2 and Q140 Huntington's Disease Mice. | 2018 Jan 9 |
2 |
| 8 |
29713895 | Mutant Huntingtin Causes a Selective Decrease in the Expression of Synaptic Vesicle Protein 2C. | 2018 Oct |
1 |
| 9 |
28270748 | Huntingtin Is Required for Neural But Not Cardiac/Pancreatic Progenitor Differentiation of Mouse Embryonic Stem Cells In vitro. | 2017 |
1 |
| 10 |
27147652 | Compromised Dopaminergic Encoding of Reward Accompanying Suppressed Willingness to Overcome High Effort Costs Is a Prominent Prodromal Characteristic of the Q175 Mouse Model of Huntington's Disease. | 2016 May 4 |
1 |
| 11 |
26048156 | Ectopic expression of the striatal-enriched GTPase Rhes elicits cerebellar degeneration and an ataxia phenotype in Huntington's disease. | 2015 Oct |
1 |
| 12 |
26691307 | Reduced Levels of Proteasome Products in a Mouse Striatal Cell Model of Huntington's Disease. | 2015 |
2 |
| 13 |
24381308 | Dexamethasone induces heat shock response and slows down disease progression in mouse and fly models of Huntington's disease. | 2014 May 15 |
1 |
| 14 |
24938402 | Impaired brain energy metabolism in the BACHD mouse model of Huntington's disease: critical role of astrocyte-neuron interactions. | 2014 Sep |
1 |
| 15 |
25268775 | The Huntington's disease-related cardiomyopathy prevents a hypertrophic response in the R6/2 mouse model. | 2014 |
1 |
| 16 |
25348412 | Altered Ca(2+) signaling in skeletal muscle fibers of the R6/2 mouse, a model of Huntington's disease. | 2014 Nov |
1 |
| 17 |
23549885 | Targeting sirtuin-1 in Huntington's disease: rationale and current status. | 2013 May |
2 |
| 18 |
21272613 | Longitudinal analysis of the behavioural phenotype in R6/1 (C57BL/6J) Huntington's disease transgenic mice. | 2012 Jun 1 |
2 |
| 19 |
22045254 | Reagents that block neuronal death from Huntington's disease also curb oxidative stress. | 2012 Jan 4 |
1 |
| 20 |
22227000 | Induced pluripotent stem cell lines from Huntington's disease mice undergo neuronal differentiation while showing alterations in the lysosomal pathway. | 2012 Apr |
1 |
| 21 |
22508027 | Multiple phenotypes in Huntington disease mouse neural stem cells. | 2012 May |
2 |
| 22 |
22589249 | Transducer of regulated CREB-binding proteins (TORCs) transcription and function is impaired in Huntington's disease. | 2012 Aug 1 |
2 |
| 23 |
22649225 | Caspase-6 activity in a BACHD mouse modulates steady-state levels of mutant huntingtin protein but is not necessary for production of a 586 amino acid proteolytic fragment. | 2012 May 30 |
3 |
| 24 |
22698685 | Tissue transglutaminase overexpression does not modify the disease phenotype of the R6/2 mouse model of Huntington's disease. | 2012 Sep |
1 |
| 25 |
21059370 | The common inhaled anesthetic isoflurane increases aggregation of huntingtin and alters calcium homeostasis in a cell model of Huntington's disease. | 2011 Feb 1 |
1 |
| 26 |
21245084 | Preferential accumulation of N-terminal mutant huntingtin in the nuclei of striatal neurons is regulated by phosphorylation. | 2011 Apr 1 |
3 |
| 27 |
22179316 | Sirt1 mediates neuroprotection from mutant huntingtin by activation of the TORC1 and CREB transcriptional pathway. | 2011 Dec 18 |
2 |
| 28 |
20519964 | Clearance of mutant huntingtin. | 2010 Jul |
3 |
| 29 |
21044321 | Lack of interleukin-1 type 1 receptor enhances the accumulation of mutant huntingtin in the striatum and exacerbates the neurological phenotypes of Huntington's disease mice. | 2010 Nov 2 |
6 |
| 30 |
19860865 | Genetic and pharmacological inhibition of calcineurin corrects the BDNF transport defect in Huntington's disease. | 2009 Oct 27 |
1 |
| 31 |
19864571 | Intrabody gene therapy ameliorates motor, cognitive, and neuropathological symptoms in multiple mouse models of Huntington's disease. | 2009 Oct 28 |
2 |
| 32 |
17947297 | HYPK, a Huntingtin interacting protein, reduces aggregates and apoptosis induced by N-terminal Huntingtin with 40 glutamines in Neuro2a cells and exhibits chaperone-like activity. | 2008 Jan 15 |
4 |
| 33 |
18537673 | A critical evaluation of adenosine A2A receptors as potentially "druggable" targets in Huntington's disease. | 2008 |
1 |
| 34 |
18558632 | Accumulation of N-terminal mutant huntingtin in mouse and monkey models implicated as a pathogenic mechanism in Huntington's disease. | 2008 Sep 1 |
1 |
| 35 |
18650014 | Inhibition of neurite outgrowth and promotion of cell death by cytoplasmic soluble mutant huntingtin stably transfected in mouse neuroblastoma cells. | 2008 Sep 5 |
1 |
| 36 |
17272267 | Systematic uncovering of multiple pathways underlying the pathology of Huntington disease by an acid-cleavable isotope-coded affinity tag approach. | 2007 May |
2 |
| 37 |
17396163 | Mechanisms of copper ion mediated Huntington's disease progression. | 2007 Mar 28 |
1 |
| 38 |
17708681 | Unbiased gene expression analysis implicates the huntingtin polyglutamine tract in extra-mitochondrial energy metabolism. | 2007 Aug |
1 |
| 39 |
17925440 | A small-molecule therapeutic lead for Huntington's disease: preclinical pharmacology and efficacy of C2-8 in the R6/2 transgenic mouse. | 2007 Oct 16 |
1 |
| 40 |
16403806 | Deletion of the triplet repeat encoding polyglutamine within the mouse Huntington's disease gene results in subtle behavioral/motor phenotypes in vivo and elevated levels of ATP with cellular senescence in vitro. | 2006 Feb 15 |
2 |
| 41 |
16443291 | Behaviour changes in a transgenic model of Huntington's disease. | 2006 Apr 25 |
1 |
| 42 |
17018277 | Transcriptional repression of PGC-1alpha by mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration. | 2006 Oct 6 |
1 |
| 43 |
17027958 | Hippi is essential for node cilia assembly and Sonic hedgehog signaling. | 2006 Dec 15 |
1 |
| 44 |
15654337 | Polyglutamine expansion of huntingtin impairs its nuclear export. | 2005 Feb |
1 |
| 45 |
16137562 | Early behavioral deficits in R6/2 mice suitable for use in preclinical drug testing. | 2005 Oct |
1 |
| 46 |
15190011 | Nuclear-targeting of mutant huntingtin fragments produces Huntington's disease-like phenotypes in transgenic mice. | 2004 Aug 1 |
3 |
| 47 |
15280037 | Identification of ubiquitin-interacting proteins in purified polyglutamine aggregates. | 2004 Jul 30 |
1 |
| 48 |
12554681 | Mismatch repair gene Msh2 modifies the timing of early disease in Hdh(Q111) striatum. | 2003 Feb 1 |
2 |
| 49 |
12657678 | Mutant huntingtin causes context-dependent neurodegeneration in mice with Huntington's disease. | 2003 Mar 15 |
4 |
| 50 |
12969246 | Inclusion formation in Huntington's disease R6/2 mouse muscle cultures. | 2003 Oct |
1 |