Results 51 to 60 of about 2,441,926 (254)
Type I interferons modulate autophagy to shape gemcitabine response in pancreatic cancer cells
FEBS Open Bio, EarlyView.Type I interferons differentially modulate autophagy and the response of pancreatic cancer cells to gemcitabine. IFNα2b stimulates autophagic flux and protects cells from gemcitabine‐induced cell death, contributing to chemoresistance. In contrast, IFNβ1a inhibits autophagosome formation and enhances gemcitabine‐induced cell death, resulting in ...
Lucy E. Bonilla +10 more
wiley +1 more source
Fabry Disease: Molecular Basis, Pathophysiology, Diagnostics and Potential Therapeutic Directions
Biomolecules, 2021 Fabry disease (FD) is a lysosomal storage disorder (LSD) characterized by the deficiency of α-galactosidase A (α-GalA) and the consequent accumulation of toxic metabolites such as globotriaosylceramide (Gb3) and globotriaosylsphingosine (lysoGb3).
Ken Kok +5 more
doaj +1 more source
, 2014 Enzyme replacement therapy is currently the only approved treatment for Pompe disease, due to acid α-glucosidase deficiency. Clinical efficacy of this approach is variable, and more effective therapies are needed.
Della Casa R +33 more
core +1 more source
Chronobiology of Cancer: How Aging Fuels Oncogenesis at the Molecular Level
Aging and Cancer, EarlyView.This graphical abstract illustrates the key biological pathways linking aging with cancer development and progression. In the upper left, cumulative exposure to ultraviolet radiation, toxins, and reactive oxygen species (ROS) causes DNA damage and genomic instability, whereas age‐related decline in repair mechanisms, such as ATM/ATR, BER, and NER ...
Anu Singh, Aroonima Misra, Sufian Zaheer
wiley +1 more source
, 2016 Exploiting “chaperone molecule” to navigate the successful assembly energy landscapes has been extensively used in biological systems, whereas in artifical supramolecular systems the “chaperone-assisted” assembly strategy to be used for the synthesis of ...
Jingwei Ji (2616013) +7 more
core +1 more source
Annals of Clinical and Translational Neurology, EarlyView.ABSTRACT Objective Variants in SLC6A1, encoding the GABA transporter 1 (GAT‐1), cause epilepsy, autism spectrum disorder, and developmental delay via loss of GABA uptake, impaired trafficking, and ER retention. We previously found that 4‐Phenylbutyrate (PBA), an FDA‐approved drug, restores GABA uptake and reduces seizures in SLC6A1‐related disorders ...
Melissa B. DeLeeuw +5 more
wiley +1 more source
Chaperone-like Activity of Calnuc Prevents Amyloid Aggregation
, 2017 Calnuc is a ubiquitously expressed protein of the EF-hand Ca2+-binding superfamily. Previous studies have implicated it in Ca2+-sensitive physiological processes, whereas details of its function and involvement in human diseases are lacking. Drawing upon
Gopala Krishna Aradhyam (3594527) +1 more
core +1 more source
Annals of Clinical and Translational Neurology, EarlyView.ABSTRACT Multisystemic smooth muscle dysfunction syndrome (MSMDS) is an ultra‐rare, ACTA2‐related disorder characterized by severe cerebrovascular disease, aortic aneurysms, and smooth muscle dysfunction. Using molecular dynamics simulations and in silico drug screening, we identified that sapropterin dihydrochloride (Kuvan) is a candidate capable of ...
Moran Hausman‐Kedem +9 more
wiley +1 more source
Prohibitin participates in the HIRA complex to promote cell metastasis in breast cancer cell lines
FEBS Open Bio, 2020 Prohibitin (PHB) is a highly conserved, ubiquitously expressed, multifunctional protein with a well‐characterized function as a chaperone‐stabilizing mitochondrial proteins.
Xiaoqing Huang, Jinji Liu, Qinghui Ma
doaj +1 more source
Advanced Science, EarlyView.This study reveals a copper‐driven mechanism of COPD acute exacerbations mediated by the TNF‐α/ATP7A axis. TNF‐α suppresses the copper transporter ATP7A via competitive NF‐κB/CREB1/CBP signaling, resulting in intracellular copper accumulation, mitochondrial oxidative stress, and epithelial barrier disruption.
Xinru Xiao +7 more
wiley +1 more source