Results 41 to 50 of about 19,163 (188)

PolyG Fibrils Coalesce Into Nuclear Ribbons That Engage Proteostasis Machinery in Neuronal Intranuclear Inclusion Disease

Advanced Science, EarlyView.
In NIID, expanded NOTCH2NLC repeats give rise to nuclear polyG inclusions. Tracer‐guided in situ cryo‐electron tomography enables cross‐scale structural analysis from mouse brain to native neuronal nuclei, revealing dense‐core/peripheral‐halo inclusions built from compact polyG ribbons.
Hui Dong, Yongcheng Pan, Zhiyao Tang, Yuxuan Yao, Guicong Zhang, Junpu Wang, Haonan Xiao, Yun Tian, Beisha Tang, Dan Li, Qiang Guo, Ruijun Tian, Qiong Liu, Cong Liu   +13 more
wiley   +1 more source

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

Engineering Antibodies into Targeted Chimeras: From Recognition Modules to Programmable Degraders

Advanced Science, EarlyView.
Antibody can be engineered into diverse lysosome‐targeting chimeras that convert target recognition into active degradation. By coupling antibody binding with recruited receptors, encoded domains, conjugated ligands, or nanoparticle scaffolds, these antibody‐associated targeted chimeras (AbTACs) platforms direct extracellular and membrane proteins ...
Kaige Chen, Quanyin Hu
wiley   +1 more source

Inhibition of mitochondrial protein import by mutant huntingtin [PDF]

Nature Neuroscience, 2014
Mitochondrial dysfunction is associated with neuronal loss in Huntington's disease (HD), a neurodegenerative disease caused by an abnormal polyglutamine expansion in huntingtin (Htt). However, the mechanisms linking mutant Htt and mitochondrial dysfunction in HD remain unknown.
Hiroko, Yano, Sergei V, Baranov, Oxana V, Baranova, Jinho, Kim, Yanchun, Pan, Svitlana, Yablonska, Diane L, Carlisle, Robert J, Ferrante, Albert H, Kim, Robert M, Friedlander   +9 more
openaire   +2 more sources

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

Exosome‐mediated gut–brain axis signaling in neurodegenerative diseases: Mechanisms, experimental evidence, and therapeutic perspectives—A narrative review

Animal Models and Experimental Medicine, EarlyView.
The gut–brain axis is a bidirectional communication network between the intestines and brain, mediated by gut microbiota and exosomes, that regulates neuroinflammation, protein aggregation, and neuronal health processes central to neurodegenerative diseases.
Waheeb Sami Aggad, Rakesh Ghosh, Hailah M. Almohaimeed, Zuhair M. Mohammedsaleh, Fayez M. Saleh, Amany I. Almars, S. Renuka Jyothi, Rajashree Panigrahi, Ajoy Kumer, Bikram Dhara   +9 more
wiley   +1 more source

Effects of overexpression of Huntingtin proteins on mitochondrial integrity [PDF]

Human Molecular Genetics, 2008
Huntington's disease (HD) is caused by an expansion of a CAG trinucleotide sequence that encodes a polyglutamine tract in the huntingtin (Htt) protein. Expansion of the polyglutamine tract above 35 repeats causes disease, with the age of onset inversely related to the degree of expansion above this number.
Hongmin, Wang, Precious J, Lim, Mariusz, Karbowski, Mervyn J, Monteiro   +3 more
openaire   +2 more sources

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