Results 71 to 80 of about 16,136 (210)

Reduced Proteasome Degradation of HSF‐1 Shifts Protein Stress Management With Age in Caenorhabditis elegans

open access: yesAging Cell, Volume 25, Issue 2, February 2026.
Ageing C. elegans prioritises chronic protein stress protection over acute stress response by coordinating HSF‐1 activity. Decreased PBS‐7 binding in aged worms reduces HSF‐1 degradation, enhancing its ability to upregulate protective factors against accumulating protein aggregates while simultaneously suppressing its activation under acute heat shock.
Hongwei Wang   +11 more
wiley   +1 more source

The physiological and pathological effects of sphingolipid metabolism and signaling in the central nervous system

open access: yesBrain Pathology, Volume 36, Issue 1, January 2026.
Sphingolipids are vital components of cell membranes. Metabolic disruptions of sphingolipids, including ceramide and sphingosine‐1‐phosphate, are linked to neurological disorders. This article summarizes the classification, structure, and metabolic processes of sphingolipids, and the physiological and pathological effects of sphingolipid metabolism and
Tian Li   +7 more
wiley   +1 more source

Polyglutamine Repeat Length-Dependent Proteolysis of Huntingtin

open access: yesNeurobiology of Disease, 2002
Amino-terminal fragments of huntingtin, which contain the expanded polyglutamine repeat, have been proposed to contribute to the pathology of Huntington's disease (HD).
Banghua Sun   +20 more
doaj   +1 more source

TDP‐43 proteinopathies and neurodegeneration: insights from Caenorhabditis elegans models

open access: yesThe FEBS Journal, Volume 293, Issue 2, Page 348-384, January 2026.
The manuscript explores structural and functional features of TDP‐43 and its worm homologue, TDP‐1, highlighting conserved and divergent structural and functional features. Using genetically engineered C. elegans models, key pathological features of TDP‐43 proteinopathies—including aggregation, neurodegeneration, and motor deficits—are recapitulated ...
Ghulam Jeelani Pir   +7 more
wiley   +1 more source

Research Progress on Idebenone in Neurodegenerative Diseases

open access: yesAGING MEDICINE, Volume 8, Issue 6, Page 624-633, December 2025.
The study not only summarizes the biological properties of Idebenone (IDE), including its role in improving mitochondrial function and antioxidative stress, but also explores its clinical research progress in various neurodegenerative diseases, providing a new perspective for the treatment of these disorders.
Yanqing Zhang   +10 more
wiley   +1 more source

The Role and Therapeutic Potential of the cGAS‐STING Signaling Pathway in Alzheimer's Disease

open access: yesBrain and Behavior, Volume 15, Issue 12, December 2025.
This work reveals the relationship between the cGAS‐STING signaling pathway and the pathological mechanisms of Alzheimer's disease.The role of the cGAS‐STING signaling pathway in AD pathology. AD leads to the production of neutrophil extracellular traps (NETs), endoplasmic reticulum (ER) stress, and the release of mitochondrial DNA (mtDNA) and nuclear ...
Xue Li, Wei Gao, Qiuyan Ye, Honglin Li
wiley   +1 more source

Antagonistic effect of cyclin-dependent kinases and a calcium-dependent phosphatase on polyglutamine-expanded androgen receptor toxic gain of function [PDF]

open access: gold, 2023
Diana Piol   +21 more
openalex   +1 more source

Antisense oligonucleotides targeting valosin‐containing protein ameliorate muscle pathology and molecular defects in cell and mouse models of multisystem proteinopathy

open access: yesClinical and Translational Medicine, Volume 15, Issue 12, December 2025.
Multisystem proteinopathy 1 (MSP1), caused by gain‐of‐function VCP variants, leads to multisystem degeneration. Using VCP patient‐derived hiPSCs, skeletal muscle progenitor cells were generated to evaluate antisense oligonucleotide (ASO) therapy.
Pallabi Pal   +14 more
wiley   +1 more source

ATRX: From Chromatin Remodeling to Disease

open access: yesgenesis, Volume 63, Issue 6, December 2025.
ABSTRACT Chromatin remodeling proteins are evolutionarily conserved factors involved in a wide range of biological processes. In this review, we describe ATRX, a chromatin remodeling protein belonging to the SWI/SNF2 family. Its association with different protein complexes, and its roles in embryonic development, sexual differentiation, as well as ...
Mauro Magaña‐Acosta   +1 more
wiley   +1 more source

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

open access: yeseLife, 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   +14 more
doaj   +1 more source

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