Results 91 to 100 of about 40,748 (225)

Identification of hepta-histidine as a candidate drug for Huntington's disease by in silico-in vitro- in vivo-integrated screens of chemical libraries. [PDF]

, 2016
We identified drug seeds for treating Huntington's disease (HD) by combining in vitro single molecule fluorescence spectroscopy, in silico molecular docking simulations, and in vivo fly and mouse HD models to screen for inhibitors of abnormal ...
Chen, Xigui, Finkbeiner, Steven, Fujita, Kyota, Homma, Hidenori, Ikura, Teikichi, Imamura, Tomomi, Ito, Nobutoshi, Kaye, Julia, Mao, Ying, Motoki, Kazumi, Nakabayashi, Makoto, Okano, Hideyuki, Okazawa, Hitoshi, Tagawa, Kazuhiko, Tamura, Takuya, Taniguchi, Juliana Bosso, Tomii, Kentaro, Yamada, Kazunori   +17 more
core   +2 more sources

Rapid generation of prion disease models using AAV‐delivered PrP variants in knockout mice

Brain Pathology, EarlyView.
We developed a rapid AAV‐based system to generate prion disease models in weeks rather than months. Following systemic AAV9P31 delivery of modified PrP to knockout mice, we achieved brain‐wide expression and successful propagation of both classical (RML) and atypical (GSS‐A117V) prion strains.
Maitena San‐Juan‐Ansoleaga, Eva Fernández‐Muñoz, Jorge M. Charco, Enric Vidal, Diego Herrero‐Martínez, Josu Galarza‐Ahumada, Cristina Sampedro‐Torres‐Quevedo, Samanta Giler, Mariví Geijo, Gloria González‐Aseguinolaza, Hasier Eraña, Joaquín Castilla   +11 more
wiley   +1 more source

Mitochondrial dynamics–fusion, fission, movement, and mitophagy–in neurodegenerative diseases [PDF]

, 2009
Neurons are metabolically active cells with high energy demands at locations distant from the cell body. As a result, these cells are particularly dependent on mitochondrial function, as reflected by the observation that diseases of mitochondrial ...
Alexander, Baloyannis, Banchs, Bossy-Wetzel, Brockmann, Cassidy-Stone, Chang, Chen, Cho, Clark, D. C. Chan, de Brito, Delettre, Dodson, Exner, Fransson, Frezza, Gispert, H. Chen, Hollenbeck, Kim, Kim, Li, Liot, Lustbader, Macaskill, Meeusen, Milone, Mortiboys, Okamoto, Orr, Park, Parone, Popp, Reis, Suen, Ten Dijke, Trushina, Wang, Waterham, Wood-Kaczmar, Zuchner, Z  chner   +42 more
core   +3 more sources

The DNA/RNA autophagy protein SIDT2 as a novel neuropathological hallmark in Huntington disease

Brain Pathology, EarlyView.
SIDT2‐immunoreactive inclusions are observed in the striatum, cerebral cortex, and hypothalamus in HD cases with different Vonsattel grades, and the frequency of SIDT2‐immunoreactive inclusions is associated with longer CAG repeats in the huntingtin gene.
Sanaz Gabery, Sofia Bergh, Chrisovalantou Huridou, Rachel Y. Cheong, Barbara Baldo, Paul Günther Scheunemann, Marie‐Louisa Schoebel, Linda Holmquist Mengelbier, Elisabet Englund, Catriona McLean, Carsten Saft, Deniz Kirik, Maria Björkqvist, Glenda Halliday, Elisabeth Petrasch‐Parwez, Huu Phuc Nguyen, Jonasz Jeremiasz Weber, Åsa Petersén   +17 more
wiley   +1 more source

MeCP2: a novel Huntingtin interactor [PDF]

Human Molecular Genetics, 2013
Transcriptional dysregulation has been proposed to play a major role in the pathology of Huntington's disease (HD). However, the mechanisms that cause selective downregulation of target genes remain unknown. Previous studies have shown that mutant huntingtin (Htt) protein interacts with a number of transcription factors thereby altering transcription ...
McFarland KN, Huizenga MN, Darnell SB, Sangrey GR, Berezovska O, Cha J-HJ, Outeiro TF, Sadri-Vakili G   +7 more
openaire   +4 more sources

Reduction of mutant huntingtin accumulation and toxicity by lysosomal cathepsins D and B in neurons

Molecular Neurodegeneration, 2011
Background Huntington's disease is caused by aggregation of mutant huntingtin (mHtt) protein containing more than a 36 polyQ repeat. Upregulation of macroautophagy was suggested as a neuroprotective strategy to degrade mutant huntingtin.
Ouyang Xiaosen, Liang Qiuli, Schneider Lonnie, Zhang Jianhua   +3 more
doaj   +1 more source

A Systems Biology Approach towards Deciphering the Unfolded Protein Response in Huntington's Disease [PDF]

, 2012
Although the disease causing gene huntingtin has been known for some time, the exact cause of neuronal cell death during _Huntington's disease_ (HD) remains unknown.
Kameshwar R. Ayasolla, Matthias E. Futschik, Ravi K. R. Kalathur   +2 more
core   +2 more sources

Clinical‐Grade Human Induced Pluripotent Stem Cell‐Derived Neural Precursor Cells Restore Motor Function and Preserve Striatal Integrity in a Quinolinic Acid‐Lesioned Rat Model of Huntington's Disease

Cell Proliferation, EarlyView.
Clinical‐grade HLA‐homozygous iPSC‐derived neural precursor cells restore motor function, rebuild striatal circuitry and reduce neuroinflammation in QA‐lesioned rats. These findings demonstrate robust neuronal replacement and microenvironment modulation, supporting their potential as a regenerative therapy for Huntington's disease.
Hyeonjoong Jeon, Il‐Shin Lee, Sanghun Lee, Hyo Chang Park, Beomsoo Kim, Hyun Sook Kim, Jihwan Song   +6 more
wiley   +1 more source

Levels of mutant huntingtin influence the phenotypic severity of Huntington disease in YAC128 mouse models

Neurobiology of Disease, 2006
Huntington disease (HD) is a devastating neuropsychiatric disease caused by expansion of a trinucleotide repeat (CAG) in the HD gene. Neuropathological changes include the appearance of N-terminal huntingtin fragments, decreased brain weight and ...
Rona K. Graham, Elizabeth J. Slow, Yu Deng, Nagat Bissada, Ge Lu, Jacqueline Pearson, Jacqueline Shehadeh, Blair R. Leavitt, Lynn A. Raymond, Michael R. Hayden   +9 more
doaj   +1 more source

Reduced Fractalkine Levels Lead to Striatal Synaptic Plasticity Deficits in Huntington’s Disease

Frontiers in Cellular Neuroscience, 2020
Huntington’s disease (HD) is an inherited neurodegenerative disorder in which the striatum is the most affected brain region. Although a chronic inflammatory microglial reaction that amplifies disease progression has been described in HD patients, some ...
Anya Kim, Anya Kim, Anya Kim, Anya Kim, Esther García-García, Esther García-García, Esther García-García, Esther García-García, Marco Straccia, Marco Straccia, Marco Straccia, Marco Straccia, Marco Straccia, Andrea Comella-Bolla, Andrea Comella-Bolla, Andrea Comella-Bolla, Andrea Comella-Bolla, Andrea Comella-Bolla, Andrés Miguez, Andrés Miguez, Andrés Miguez, Andrés Miguez, Andrés Miguez, Mercè Masana, Mercè Masana, Mercè Masana, Mercè Masana, Jordi Alberch, Jordi Alberch, Jordi Alberch, Jordi Alberch, Jordi Alberch, Josep M. Canals, Josep M. Canals, Josep M. Canals, Josep M. Canals, Josep M. Canals, Manuel J. Rodríguez, Manuel J. Rodríguez, Manuel J. Rodríguez, Manuel J. Rodríguez   +40 more
doaj   +1 more source