A novel rhesus macaque model of Huntington’s disease recapitulates key neuropathological changes along with motor and cognitive decline by Alison R Weiss, William A Liguore, Kristin Brandon, Xiaojie Wang, Zheng Liu, Jacqueline S Domire, Dana Button, Sathya Srinivasan, Christopher D Kroenke, Jodi L McBride

“…This modeling strategy results in expression of mutant huntingtin protein (mHTT) and aggregate formation in the injected brain regions, as well as dozens of other cortical and subcortical brain regions affected in human HD patients. …”
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Immunohistochemical Expression Pattern of FGFR1, FGFR2, RIP5, and HIP2 in Developing and Postnatal Kidneys of <i>Dab1^−/−</i> (<i>yotari</i>) Mice by Nela Kelam, Anita Racetin, Yu Katsuyama, Katarina Vukojević, Sandra Kostić

“…This study aimed to explore how <i>Dab1</i> gene functional silencing influences the spatial and temporal expression patterns of fibroblast growth factor receptor 1 (FGFR1), fibroblast growth factor receptor 2 (FGFR2), receptor-interacting protein kinase 5 (RIP5), and huntingtin-interacting protein 2 (HIP2) in the developing and postnatal kidneys of the <i>yotari</i> mice as potential determinants of normal kidney formation and function. …”
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ABHD11-AS1: An Emerging Long Non-Coding RNA (lncRNA) with Clinical Significance in Human Malignancies by Upendarrao Golla, Kishore Sesham, Siva Dallavalasa, Naresh Kumar Manda, Sambamoorthy Unnam, Arun Kumar Sanapala, Sharada Nalla, Susmitha Kondam, Rajesh Kumar

“…In contrast, the mouse homolog of AHD11-AS1 (Abhd11os) overexpression had exhibited neuroprotective effects against mutant huntingtin-induced toxicity. Considering the emerging research reports, the authors attempted in this first review on ABHD11-AS1 to summarize and highlight its oncogenic potential and clinical significance in different human cancers. …”
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Small Molecule Pytren-4QMn Metal Complex Slows down Huntington’s Disease Progression in Male zQ175 Transgenic Mice by Marián Merino, Sonia González, Mª Carmen Tronch, Ana Virginia Sánchez-Sánchez, Mª Paz Clares, Antonio García-España, Enrique García-España, José L. Mullor

“…It is caused by an autosomal dominant mutation consisting of expansions of trinucleotide repeats that translate into poly-glutamine enlarged mutant huntingtin proteins (mHTT), which are particularly deleterious in brain tissues. …”
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Subcortical T1-Rho MRI Abnormalities in Juvenile-Onset Huntington’s Disease by Alexander V. Tereshchenko, Jordan L. Schultz, Ansley J. Kunnath, Joel E. Bruss, Eric A. Epping, Vincent A. Magnotta, Peg C. Nopoulos

“…Huntington’s disease (HD) is a fatal neurodegenerative disease caused by the expansion of cytosine-adenine-guanine (CAG) repeats in the <i>huntingtin</i> gene. An increased CAG repeat length is associated with an earlier disease onset. …”
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Naïve Huntington’s disease microglia mount a normal response to inflammatory stimuli but display a partially impaired development of innate immune tolerance that can be counteracte... by Noam Steinberg, Danny Galleguillos, Asifa Zaidi, Melanie Horkey, Simonetta Sipione

“…HD is a genetic condition caused by a mutation that affects the folding and function of huntingtin (HTT). Signs of microglia activation have been observed in HD patients even before the onset of symptoms. …”
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Chaperone-mediated autophagy in neuronal dendrites utilizes activity-dependent lysosomal exocytosis for protein disposal by Katarzyna M. Grochowska, Marit Sperveslage, Rajeev Raman, Antonio V. Failla, Dawid Głów, Christian Schulze, Laura Laprell, Boris Fehse, Michael R. Kreutz

“…This mechanism enables local disposal of aggregation-prone proteins like TDP-43 and huntingtin.…”
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A novel and accurate full-length HTT mouse model for Huntington’s disease by Sushila A Shenoy, Sushuang Zheng, Wencheng Liu, Yuanyi Dai, Yuanxiu Liu, Zhipeng Hou, Susumu Mori, Yi Tang, Jerry Cheng, Wenzhen Duan, Chenjian Li

“…At 11 months, BAC226Q mice showed significant reduced life span, gradual weight loss and exhibited neuropathology including significant brain atrophy specific to striatum and cortex, striatal neuronal death, widespread huntingtin inclusions, and reactive pathology. Therefore, the novel BAC226Q mouse accurately recapitulating robust, age-dependent, progressive HD-like phenotypes will be a valuable tool for studying disease mechanisms, identifying biomarkers, and testing gene-targeting therapeutic approaches for HD.…”
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Reconstituting Corticostriatal Network on-a-Chip Reveals the Contribution of the Presynaptic Compartment to Huntington’s Disease by Amandine Virlogeux, Eve Moutaux, Wilhelm Christaller, Aurélie Genoux, Julie Bruyère, Elodie Fino, Benoit Charlot, Maxime Cazorla, Frédéric Saudou

“…Keywords: microchambers, microfluidics, huntingtin, axonal and dendritic transport, BDNF, mitochondria, glutamate, TrkB, synapse, GCaMP6f…”
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Novel EAAT2 activators improve motor and cognitive impairment in a transgenic model of Huntington’s disease by Akanksha Bhatnagar, Visha Parmar, Visha Parmar, Nicholas Barbieri, Nicholas Barbieri, Frank Bearoff, Felice Elefant, Sandhya Kortagere

“…In this study, we test the neuroprotective abilities of these novel EAAT2 activators in vivo using the robust Drosophila HD transgenic model expressing human huntingtin gene with expanded repeats (Htt128Q).ResultsAll three compounds significantly restored motor function impaired under HD pathology over a wide dose range. …”
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Investigating toxicity in Drosophila models of Huntington's Disease and Huntington's Disease-Like 2 by Krench, Megan Attardo

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A Drosophila model of Huntington disease-like 2 exhibits nuclear toxicity and distinct pathogenic mechanisms from Huntington disease by Krench, Megan A., Cho, Richard W., Littleton, J. Troy

“…HD is caused by a CAG repeat expansion in the huntingtin (HTT) gene, while HDL2 results from an expansion at the junctophilin 3 (JPH3) locus. …”
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Treatment with JQ1, a BET bromodomain inhibitor, is selectively detrimental to R6/2 Huntington’s disease mice by Kedaigle, Amanda J, Reidling, Jack C, Lim, Ryan G, Adam, Miriam, Wu, Jie, Wassie, Brook T., Stocksdale, Jennifer T, Casale, Malcolm S, Fraenkel, Ernest, Thompson, Leslie M

“…Dysregulation of HD-related pathways in striatum was exacerbated by JQ1 in R6/2 mice, but not in NTs, and JQ1 caused a corresponding increase in the formation of a mutant huntingtin protein-dependent high molecular weight species associated with pathogenesis. …”
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Caldag-Gefi Down-Regulation in the Striatum as a Neuroprotective Change in Huntington's Disease. by Dunn, Denise E., Merali, Farhan I., Woodman, Ben, Yim, Michael, Borkowska, Anna E., Frosch, Matthew P., Bates, Gillian P., Lo, Donald C., Crittenden, Jill R, Housman, David E, Graybiel, Ann M

“…Huntingtin protein (Htt) is ubiquitously expressed, yet Huntington’s disease (HD), a fatal neurologic disorder produced by expansion of an Htt polyglutamine tract, is characterized by neurodegeneration that occurs primarily in the striatum and cerebral cortex. …”
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SIRT2 Ablation Has No Effect on Tubulin Acetylation in Brain, Cholesterol Biosynthesis or the Progression of Huntington's Disease Phenotypes In Vivo by Bobrowska, Anna, Donmez Yalcin, Gizem, Weiss, Andreas, Guarente, Leonard Pershing, Bates, Gillian P.

“…Furthermore, we observed no change in aggregate load or levels of soluble mutant huntingtin transprotein. Intriguingly, neither the constitutive genetic loss nor acute pharmacological inhibition of SIRT2 affected the expression of cholesterol biosynthesis enzymes in the context of HD. …”
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Targeting H3K4 trimethylation in Huntington disease by Ng, Christopher W., Yildirim, Ferah, Labadorf, Adam, Fraenkel, Ernest, Vashishtha, Malini, Kratter, Ian H., Bodai, Laszlo, Song, Wan, Lau, Alice L., Vogel-Ciernia, Annie, Troncosco, Juan, Ross, Christopher A., Bates, Gillian P., Krainc, Dimitri, Sadri-Vakili, Ghazaleh, Finkbeiner, Steven, Marsh, J. Lawrence, Thompson, Leslie M., Wasylenko, Theresa Anne, Housman, David E

“…Reducing the levels of the H3K4 demethylase SMCX/Jarid1c in primary neurons reversed down-regulation of key neuronal genes caused by mutant Huntingtin expression. Finally, reduction of SMCX/Jarid1c in primary neurons from BACHD mice or the single Jarid1 in a Drosophila HD model was protective. …”
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Intrinsic mutant HTT-mediated defects in oligodendroglia cause myelination deficits and behavioral abnormalities in Huntington disease by Bardile, Costanza Ferrari, Garcia-Miralles, Marta, Caron, Nicholas S., Rayan, Nirmala Arul, Langley, Sarah Raye, Harmston, Nathan, Rondelli, Ana Maria, Teo, Roy Tang Yi, Waltl, Sabine, Anderson, Lisa M., Bae, Han-Gyu, Jung, Sangyong, Williams, Anna, Prabhakar, Shyam, Petretto, Enrico, Hayden, Michael R., Pouladi, Mahmoud A.

“…We further show that selective inactivation of mutant huntingtin (mHTT) in the NG2+ oligodendrocyte progenitor cell population prevented myelin abnormalities and certain behavioral deficits in HD mice. …”
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The role of transcription and RNA/DNA structures in the pathology of Huntington's disease by Beghè, C

“…<p>Huntington’s Disease (HD) is a repeat expansion disease where a CAG-triplet expansion in the first exon 1 of the huntingtin (<em>HTT</em>) gene leads to the production of a mutant HTT (mHTT) protein. mHTT forms toxic aggregates that alter mitochondrial function, impair protein degradation and lead to neuronal cell death, causing degeneration of medium spiny neurons (MSNs) in the brains of HD patients. …”

Characterization of DnaJB6: a molecular chaperone implicated in LGMD type 1D by Thomasen, F

“…DnaJB6 is a human J-protein shown to suppress aggregation of misfolded disease-related proteins such as huntingtin, aβ42 and α-synuclein, either through the HSP70 pathway or independently. …”

Integrated analysis on transcriptome and behaviors defines HTT repeat-dependent network modules in Huntington's disease by Lulin Huang, Li Fang, Qian Liu, Abolfazl Doostparast Torshizi, Kai Wang

“…Huntington's disease (HD) is caused by a CAG repeat expansion in the huntingtin (HTT) gene. Knock-in mice carrying a CAG repeat-expanded Htt will develop HD phenotypes. …”
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