Results 41 to 50 of about 19,992 (185)

Raft-like microdomains play a key role in mitochondrial impairment in lymphoid cells from patients with Huntington's disease

Journal of Lipid Research, 2012
Huntington's disease (HD) is a genetic neurodegenerative disease characterized by an exceedingly high number of contiguous glutamine residues in the translated protein, huntingtin (Htt).
Laura Ciarlo, Valeria Manganelli, Paola Matarrese, Tina Garofalo, Antonella Tinari, Lucrezia Gambardella, Matteo Marconi, Maria Grasso, Roberta Misasi, Maurizio Sorice, Walter Malorni   +10 more
doaj   +1 more source

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

Therapeutic Antisense Targeting of Huntingtin [PDF]

DNA and Cell Biology, 2020
Antisense oligonucleotides (ASOs) are a relatively new therapeutic entity that utilizes short chemically modified strands of DNA in targeted interactions with RNA to modulate the type or amount of resultant protein. This brief review summarizes the preclinical, translational, and early clinical development of an ASO designed to reduce the production of
Smith, AV, Tabrizi, SJ
openaire   +3 more sources

PIN1 Modulates Huntingtin Levels and Aggregate Accumulation: An In vitro Model

Frontiers in Cellular Neuroscience, 2017
Huntington's disease (HD) is a dominantly inherited neurodegenerative disorder characterized by a polyglutamine expansion within the N-terminal region of huntingtin protein (HTT).
Alisia Carnemolla, Silvia Michelazzi, Elena Agostoni   +2 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

Ubiquitin-modifying enzymes in Huntington’s disease

Frontiers in Molecular Biosciences, 2023
Huntington’s disease (HD) is a neurodegenerative disorder caused by a CAG repeat expansion in the N-terminus of the HTT gene. The CAG repeat expansion translates into a polyglutamine expansion in the mutant HTT (mHTT) protein, resulting in intracellular ...
Karen A. Sap, Karlijne W. Geijtenbeek, Sabine Schipper-Krom, Arzu Tugce Guler, Eric A. Reits   +4 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

Wild-Type Huntingtin Reduces the Cellular Toxicity of Mutant Huntingtin In Vivo [PDF]

The American Journal of Human Genetics, 2001
We have developed yeast artificial chromosome (YAC) transgenic mice expressing normal (YAC18) and mutant (YAC46 or YAC72) human huntingtin (htt), in a developmental- and tissue-specific manner, that is identical to endogenous htt. YAC72 mice develop selective degeneration of medium spiny projection neurons in the lateral striatum, similar to what is ...
Leavitt, Blair R., Guttman, Julian A., Hodgson, J.Graeme, Kimel, Gil H., Singaraja, Roshni, Vogl, A.Wayne, Hayden, Michael R.   +6 more
openaire   +2 more sources

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

Stimulation of NeuroD activity by huntingtin and huntingtin-associated proteins HAP1 and MLK2 [PDF]

Proceedings of the National Academy of Sciences, 2003
NeuroD (ND) is a basic helix–loop–helix transcription factor important for neuronal development and survival. By using a yeast two-hybrid screen, we identified two proteins that interact with ND, huntingtin-associated protein 1 (HAP1) and mixed-lineage kinase 2 (MLK2), both of which are known to interact with huntingtin (Htt).
Edoardo, Marcora, Katherine, Gowan, Jacqueline E, Lee   +2 more
openaire   +2 more sources