Results 61 to 70 of about 21,037 (206)

Polyglutamine expansion affects huntingtin conformation in multiple Huntington’s disease models

Scientific Reports, 2017
Conformational changes in disease-associated or mutant proteins represent a key pathological aspect of Huntington’s disease (HD) and other protein misfolding diseases.
Manuel Daldin, V. Fodale, Cristina Cariulo, Lucia Azzollini, Margherita Verani, Paola Martufi, Maria Carolina Spiezia, Sean M. Deguire, Marta Cherubini, D. Macdonald, A. Weiss, A. Bresciani, J. Vonsattel, Lara Petricca, J. Marsh, S. Ginés, I. Santimone, M. Marano, H. Lashuel, F. Squitieri, A. Caricasole   +20 more
semanticscholar   +1 more source

Targeting signaling pathways in neurodegenerative diseases: Quercetin's cellular and molecular mechanisms for neuroprotection

Animal Models and Experimental Medicine, Volume 8, Issue 5, Page 798-818, May 2025.
Quercetin regulates signaling pathways in neurodegenerative diseases, including NF‐κB, sirtuins, and PI3K/Akt. Studies show quercetin improves symptoms and pathology in neurodegenerative models. The study aims to incorporate laboratory research into practical medical treatment, focusing on quercetin's neuroprotective effects and optimal dosage ...
Md. Rezaul Islam, Md. Ibrahim Khalil Al‐Imran, Mehrukh Zehravi, Sherouk Hussein Sweilam, Mohammad Rakib Mortuza, Jeetendra Kumar Gupta, Thukani Sathanantham Shanmugarajan, Kadirvel Devi, Tanuja Tummala, Mohammed Ali Alshehri, Kalirajan Rajagopal, Mohammed Asiri, Irfan Ahmad, Talha Bin Emran   +13 more
wiley   +1 more source

Proteolytic Cleavage of Polyglutamine Disease-Causing Proteins: Revisiting the Toxic Fragment Hypothesis.

Current pharmaceutical design, 2017
Proteolytic cleavage has been implicated in the pathogenesis of diverse neurodegenerative diseases involving abnormal protein accumulation. Polyglutamine diseases are a group of nine hereditary disorders caused by an abnormal expansion of repeated ...
Carlos A. Matos, L. P. Almeida, Clévio Nóbrega   +2 more
semanticscholar   +1 more source

Adult Neurogenesis in the Subventricular Zone of Patients with Huntington's and Parkinson's Diseases and following Long‐Term Treatment with Deep Brain Stimulation

Annals of Neurology, Volume 97, Issue 5, Page 894-906, May 2025.
Objective Parkinson's and Huntington's diseases are characterized by progressive neuronal loss. Previous studies using human postmortem tissues have shown the impact of neurodegenerative disorders on adult neurogenesis. The extent to which adult neural stem cells are activated in the subventricular zone and whether therapeutic treatments such as deep ...
Marta Snapyan, Francis Desmeules, Jonathan Munro, Morgan Bérard, Stephan Saikali, Peter V. Gould, Maxime Richer, Emmanuelle Pourcher, Mélanie Langlois, Anne‐Marie Dufresne, Michel Prud'homme, Léo Cantin, André Parent, Armen Saghatelyan, Martin Parent   +14 more
wiley   +1 more source

The Ubiquitination, Disaggregation and Proteasomal Degradation Machineries in Polyglutamine Disease

Frontiers in Molecular Neuroscience, 2017
Polyglutamine disorders are chronic, progressive neurodegenerative diseases caused by expansion of a glutamine tract in widely expressed genes. Despite excellent models of disease, a well-documented clinical history and progression, and established ...
S. Nath, A. Lieberman
semanticscholar   +1 more source

Aggregate-prone proteins with polyglutamine and polyalanine expansions are degraded by autophagy.

Human Molecular Genetics, 2002
Protein conformational disorders (PCDs), such as Alzheimer's disease, Huntington's disease (HD), Parkinson's disease and oculopharyngeal muscular dystrophy, are associated with proteins that misfold and aggregate.
B. Ravikumar, R. Duden, D. Rubinsztein
semanticscholar   +1 more source

Examination of Ataxin-3 (atx-3) Aggregation by Structural Mass Spectrometry Techniques: A Rationale for Expedited Aggregation upon Polyglutamine (polyQ) Expansion

Molecular & Cellular Proteomics, 2015
Expansion of polyglutamine stretches leads to the formation of polyglutamine-containing neuronal aggregates and neuronal death in nine diseases for which there currently are no treatments or cures.
C. Scarff, Bruno Almeida, Joana S Fraga, Sandra Macedo-Ribeiro, S. Radford, A. Ashcroft   +5 more
semanticscholar   +1 more source

Structure of polyglutamine [PDF]

FEBS Letters, 2000
Bernhard Rupp, Eric Lewin Altschuler, J.A. Mazrimas, Nicholas V. Hud   +3 more
openaire   +1 more source

The Linkage of Kennedy's Neuron Disease to ARA24, the First Identified Androgen Receptor Polyglutamine Region-associated Coactivator [PDF]

, 1999
Pei‐Wen Hsiao, Din‐Lii Lin, Ryusuke Nakao, Chawnshang Chang   +3 more
openalex   +1 more source

Polyglutamine-Expanded Androgen Receptors Form Aggregates That Sequester Heat Shock Proteins, Proteasome Components and SRC-1, and Are Suppressed by the HDJ-2 Chaperone [PDF]

, 1999
David L. Stenoien, Christopher J. Cummings, Henry P. Adams, M. Mancini, Kavita Patel, George Demartino, Marco Marcelli, Nancy L. Weigel, Michael A. Mancini   +8 more
openalex   +1 more source