Results 51 to 60 of about 25,505 (239)

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

open access: yesJournal 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   +10 more
doaj   +1 more source

Hsp40 overexpression in pacemaker neurons delays circadian dysfunction in a Drosophila model of Huntington's disease

open access: yesDisease Models & Mechanisms, 2022
Circadian disturbances are early features of neurodegenerative diseases, including Huntington's disease (HD). Emerging evidence suggests that circadian decline feeds into neurodegenerative symptoms, exacerbating them.
Pavitra Prakash   +2 more
doaj   +1 more source

Multifunctional Gold Nanocluster‐Based PROTAC System for Targeted Degradation of Phosphorylated Tau and Modulation of Neuroinflammation in Alzheimer's Disease

open access: yesAdvanced Functional Materials, EarlyView.
We present a novel proteolysis‐targeting chimera (PROTAC) system conjugated to lipoic acid gold nanoclusters (PLANC), designed to degrade pTau, regulate inflammatory signaling, and effectively traverse the blood‐brain barrier (BBB). PLANC degraded pTau at various phosphorylation sites, with mechanistic studies confirming proteasome‐mediated degradation
Sarah Nevins   +9 more
wiley   +1 more source

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

open access: yesThe 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.   +6 more
openaire   +2 more sources

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

open access: yesFrontiers 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   +2 more
doaj   +1 more source

TDP‐43 Aggregation: The Healthy‐Toxic Balance of the Prion‐Like Domain

open access: yesAdvanced Science, EarlyView.
TDP‐43 function relies on a delicate balance between reversible phase‐separated states and irreversible aggregation. Under physiological conditions, TDP‐43 forms dynamic droplets and oligomers that support normal cellular functions. In pathological contexts, this balance shifts toward aberrant aggregation, leading to toxic species.
Luca Zangrando   +2 more
wiley   +1 more source

Calmodulin regulates transglutaminase 2 cross-linking of Huntingtin [PDF]

open access: yes, 2004
This is the publisher's version, also available electronically from "www.jneurosci.org".Striatal and cortical intranuclear inclusions and cytoplasmic aggregates of mutant huntingtin are prominent neuropathological hallmarks of Huntington's disease (HD ...
Ross, Christopher A.   +4 more
core   +1 more source

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

open access: yesAnimal 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   +9 more
wiley   +1 more source

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

open access: yesProceedings 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   +2 more
openaire   +2 more sources

Huntingtin-RNA complex generation for cryoEM analysis 2019/07/24

open access: yes, 2019
Project: High resolution structural analysis of HTT-nucleic acid complexes Experiment: Huntingtin-RNA complex generation for cryoEM analysis Date: 2019/07/24 Background: Huntingtin has been shown to copurify with nucleic acid material as well as bind ...
Arrowsmith, Cheryl (4928326)   +7 more
core   +1 more source

Home - About - Disclaimer - Privacy