Results 51 to 60 of about 3,376,711 (176)

The Huntington disease protein accelerates breast tumour development and metastasis through ErbB2/HER2 signalling

EMBO Molecular Medicine, 2013
In Huntington disease (HD), polyglutamine expansion in the huntingtin protein causes specific neuronal death. The consequences of the presence of mutant huntingtin in other tissues are less well understood.
Cristovão Moreira Sousa, John Russel McGuire, Morgane Sonia Thion, David Gentien, Pierre de la Grange, Sophie Tezenas du Montcel, Anne Vincent‐Salomon, Alexandra Durr, Sandrine Humbert   +8 more
doaj   +1 more source

Huntingtin Aggregate‐Responsive Autophagy Gene Circuit Mitigates Disease Pathology in R6/2 Mice

Advanced Science, EarlyView.
CD98‐mediated receptor‐mediated transcytosis enables LIP‐CD98 nanocarriers to cross the blood–brain barrier and deliver ARAA to neurons. mHTT aggregates activate the 11G–NarX sensor, initiating Auto‐P and Trans‐P signaling through the VP48–NarL relay.
Jie Zhu, Xi‐xiu Xie, Lei Li, Chen Tian, Hao‐tian Wang, Xiao‐jie Wang, Xiao‐lin Yu, Gui‐feng Zhang, Rui‐tian Liu   +8 more
wiley   +1 more source

Multimodal Characterization of Glymphatic‐Related Magnetic Resonance Imaging Markers in Huntington's Disease: A Multi‐Cohort Retrospective Study

Annals of Neurology, EarlyView.
Objective To characterize magnetic resonance imaging (MRI)‐based glymphatic surrogates in Huntington's disease (HD) using MRI measures of perivascular diffusivity and structural perivascular alterations across multiple large cohorts. Methods We analyzed 2,731 MRI sessions from 880 participants across 3 large retrospective HD cohorts.
Alexia Solomon, Zexi Wang, Francesco Sanvito, Zheng Zhou, Adys Mendizabal, Jingwen Yao   +5 more
wiley   +1 more source

Novel DNA Aptamers that Bind to Mutant Huntingtin and Modify Its Activity

Molecular Therapy: Nucleic Acids, 2018
The CAG repeat expansion that elongates the polyglutamine tract in huntingtin is the root genetic cause of Huntington’s disease (HD), a debilitating neurodegenerative disorder.
Baehyun Shin, Roy Jung, Hyejin Oh, Gwen E. Owens, Hyeongseok Lee, Seung Kwak, Ramee Lee, Susan L. Cotman, Jong-Min Lee, Marcy E. MacDonald, Ji-Joon Song, Ravi Vijayvargia, Ihn Sik Seong   +12 more
doaj   +1 more source

Huntingtin-Associated Protein 1 in Mouse Hypothalamus Stabilizes Glucocorticoid Receptor in Stress Response

Frontiers in Cellular Neuroscience, 2020
Huntingtin-associated protein 1 (Hap1) was initially identified as a brain-enriched protein that binds to the Huntington’s disease protein, huntingtin. Unlike huntingtin that is ubiquitously expressed in the brain, Hap1 is enriched in the brain with the ...
Xingxing Chen, Xingxing Chen, Xingxing Chen, Ning Xin, Yongcheng Pan, Yongcheng Pan, Louyin Zhu, Peng Yin, Qiong Liu, Qiong Liu, Weili Yang, Xingshun Xu, Shihua Li, Xiao-Jiang Li   +13 more
doaj   +1 more source

Fibrilpaint: A Class of Amyloid‐Targeting Peptides

Chemistry – A European Journal, EarlyView.
FibrilPaints are modular peptides engineered to recognize amyloid fibrils. By systematically varying charge, sequence, and termini, their binding behavior can be tuned while retaining recognition. Successful binding ″paints″ fibrils for visualization and opens routes toward targeted degradation and diagnostic applications.
Júlia Aragonès Pedrola, Françoise A. Dekker, Katrin Guttmann, Litske M. van Leeuwen, Shalini Singh, Guy Mayer, Tommaso Garfagnini, Assaf Friedler, Stefan G. D. Rüdiger   +8 more
wiley   +1 more source

The mTOR kinase inhibitor Everolimus decreases S6 kinase phosphorylation but fails to reduce mutant huntingtin levels in brain and is not neuroprotective in the R6/2 mouse model of Huntington's disease

Molecular Neurodegeneration, 2010
Background Huntington's disease (HD) is a progressive neurodegenerative disorder caused by a CAG repeat expansion within the huntingtin gene. Mutant huntingtin protein misfolds and accumulates within neurons where it mediates its toxic effects. Promoting
Frentzel Stefan, Hoyer Daniel, Betschart Claudia, Bleckmann Dorothee, Kama Jibrin A, Dorsey Kate, Chopra Vanita, Connor Teal, Fox Jonathan H, DiFiglia Marian, Paganetti Paolo, Hersch Steven M   +11 more
doaj   +1 more source

The Cytotoxicity and Clearance of Mutant Huntingtin and Other Misfolded Proteins

Cells, 2021
Protein misfolding and aggregation are implicated in many neurodegenerative diseases. One of these diseases is Huntington’s, which is caused by increased glutamine-encoding trinucleotide repeats within the Huntingtin gene.
Austin Folger, Yanchang Wang
doaj   +1 more source

ALTERED EXPRESSION AND FUNCTIONALITY OF A2A ADENOSINE RECEPTORS IN HUNTINGTON’S DISEASE AND OTHER POLYGLUTAMINE DISORDERS [PDF]

, 2009
Several studies have suggested the possible involvement of A2A adenosine receptors in the pathogenesis of neuronal disorders, including Huntington’s disease.
Vincenzi, Fabrizio
core  

Rapamycin reduces neuronal mutant huntingtin aggregation and ameliorates locomotor performance in Drosophila

Frontiers in Aging Neuroscience, 2023
Huntington’s disease (HD) is a neurodegenerative disease characterized by movement and cognitive dysfunction. HD is caused by a CAG expansion in exon 1 of the HTT gene that leads to a polyglutamine (PQ) repeat in the huntingtin protein, which aggregates ...
Jonathan R. Roth, Jonathan R. Roth, Ruan Carlos Macedo de Moraes, Brittney P. Xu, Savannah R. Crawley, Malghalara A. Khan, Girish C. Melkani   +6 more
doaj   +1 more source