Results 71 to 80 of about 14,352 (184)

Nuclear dysfunction in aging and neurodegeneration

Alzheimer's &Dementia, Volume 22, Issue 7, July 2026.
Abstract Neurodegenerative diseases are characterized by a loss of neuronal function and structure, often in a region‐specific manner. Multiple factors contribute to neuronal dysfunction and death, including pathogenic protein buildup, protein mislocalization, and inflammation. Despite extensive research, the common mechanisms driving neurodegeneration
Abbigael Aday, Jack Tiller, Adarsh Melukote, Zachary C. Walton, Anna King, Luke Castillo, Rakez Kayed, Mauro Montalbano   +7 more
wiley   +1 more source

Mesenchymal Stem Cell‐Based Therapy for Cerebellar Ataxia: From Bench to Bedside

CNS Neuroscience &Therapeutics, Volume 32, Issue 7, July 2026.
Allogeneic hMSCs transplanted across LPS, Ara‐C, and SCA2 cerebellar ataxia (CA) models suppress neuroinflammation and restore the neurotrophin axis, collectively preserving Purkinje cell integrity. These preclinical findings are being translated clinically, from a first‐in‐human case report to an ongoing Phase II/III randomized trial (NCT02540655 ...
Kyoungho Suk, Ho‐Won Lee, Sang Ryong Kim   +2 more
wiley   +1 more source

Mutant huntingtin impairs PNKP and ATXN3, disrupting DNA repair and transcription

eLife, 2019
How huntingtin (HTT) triggers neurotoxicity in Huntington’s disease (HD) remains unclear. We report that HTT forms a transcription-coupled DNA repair (TCR) complex with RNA polymerase II subunit A (POLR2A), ataxin-3, the DNA repair enzyme polynucleotide ...
Rui Gao, Anirban Chakraborty, Charlene Geater, Subrata Pradhan, Kara L Gordon, Jeffrey Snowden, Subo Yuan, Audrey S Dickey, Sanjeev Choudhary, Tetsuo Ashizawa, Lisa M Ellerby, Albert R La Spada, Leslie M Thompson, Tapas K Hazra, Partha S Sarkar   +14 more
doaj   +1 more source

Allosteric Modulation of Pathological Ataxin‐3 Aggregation: A Path to Spinocerebellar Ataxia Type‐3 Therapies

Advanced Science
Spinocerebellar ataxia type 3 (SCA3) is a rare neurodegenerative disorder caused by the expansion of a polyglutamine (polyQ) repeat in ataxin‐3 (Atx3) for which no disease‐modifying therapies are available.
Alexandra Silva, Sara Duarte‐Silva, Pedro M. Martins, Beatriz Rodrigues, Débora Serrenho, Daniela Vilasboas‐Campos, Andreia Teixeira‐Castro, Julio Vieyto‐Nuñez, Joel Mieres‐Perez, Francisco Figueiredo, Martyna Podlasiak, Tatiana Van‐der‐Kellen, Joana Fraga, James Noble, Carter Lantz, Niki Sepanj, Daniela Monteiro‐Fernandes, Sara Guerreiro, Andreia Neves‐Carvalho, Joana Pereira‐Sousa, Frank‐Gerrit Klärner, Thomas Schrader, Joseph A. Loo, Annalisa Pastore, Elsa Sanchez‐Garcia, Gal Bitan, Ana Luísa Carvalho, Patrícia Maciel, Sandra Macedo‐Ribeiro   +28 more
doaj   +1 more source

An Optimal Ubiquitin-Proteasome Pathway in the Nervous System: The Role of Deubiquitinating Enzymes

Frontiers in Molecular Neuroscience, 2014
The Ubiquitin-Proteasome Pathway (UPP), which is critical for normal function in the nervous system and is implicated in various neurological diseases, requires the small modifier protein ubiquitin to accomplish its duty of selectively degrading short ...
Gorica eRistic, Wei-Ling eTsou, Sokol V Todi   +2 more
doaj   +1 more source

Biophysical insights into how surfaces, including lipid membranes, modulate protein aggregation related to neurodegeneration

Frontiers in Neurology, 2013
There are a vast number of neurodegenerative diseases, including Alzheimer’s disease (AD), Parkinson’s disease (PD), and Huntington’s disease (HD), associated with the rearrangement of specific proteins to non-native conformations that promotes ...
Kathleen A Burke, Elizabeth A Yates, Justin eLegleiter   +2 more
doaj   +1 more source

Polyglutamine Disease: Acetyltransferases Awry [PDF]

Current Biology, 2002
Recent evidence indicates that inhibition of histone acetyltransferases may be a primary cause of cellular pathogenesis in polyglutamine diseases such as Huntington disease; the results raise the possibility that pharmacologic manipulation of protein acetylation levels could be of therapeutic benefit.
openaire   +2 more sources

Chemical Chaperones Reduce Aggregate Formation and Cell Death Caused by the Truncated Machado–Joseph Disease Gene Product with an Expanded Polyglutamine Stretch

Neurobiology of Disease, 2002
Machado–Joseph disease/spinocerebellar ataxia-3 (MJD/SCA-3) is an inherited neurodegenerative disorder caused by expansion of the polyglutamine stretch in the MJD gene-encoded protein ataxin-3.
Hideaki Yoshida, Toshihiro Yoshizawa, Futoshi Shibasaki, Shin'ichi Shoji, Ichiro Kanazawa   +4 more
doaj   +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

Enhanced Age-Dependent Motor Impairment in Males of Drosophila melanogaster Modeling Spinocerebellar Ataxia Type 1 Is Linked to Dysregulation of a Matrix Metalloproteinase

Biology
Over the past two decades, Drosophila melanogaster has proven to be successful in modeling the polyglutamine (polyQ) (caused by CAG repeats) family of neurodegenerative disorders, including the faithful recapitulation of pathological features such as ...
Emma M. Palmer, Caleb A. Snoddy, Peyton M. York, Sydney M. Davis, Madelyn F. Hunter, Natraj Krishnan   +5 more
doaj   +1 more source