Results 61 to 70 of about 16,862 (246)

Metal chaperones: a holistic approach to the treatment of AD

Frontiers in Psychiatry, 2012
As the burden of proof for the role of metal ion dysregulation in the pathogenesis of multiple CNS disorders grows, it has become important to more precisely identify and differentiate the biological effects of various pharmacological modulators of metal
Paul Anthony Adlard, Ashley Ian Bush
doaj   +1 more source

Investigation of novel pharmacological chaperones for Gaucher Disease

Journal of Molecular Graphics and Modelling, 2017
Beta-Glucocerebrosidase (GBA) is a lysosomal protein that is responsible for the hydrolysis of glycosylceramide into glucose and ceramide. Mutations in GBA lead to the accumulation of glycosylceramide in the lysosome causing an enlargement of the spleen and the liver and skeletal deformations. This disease is called Gaucher Disease.
Yilmazer, Buge, Yagci, Z. Begum, Bakar, Emre, Ozden, Burcu, Ulgen, Kutlu, Ozkirimli, Elif   +5 more
openaire   +3 more sources

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, Martín M. Ledesma, Santiago A. Behr, Matías A. Pibuel, María L. Palanek, Daniel H. Grasso, Maria N. Garcia, Juan Garona, Silvina L. Lompardía, Elida Álvarez, Daniela L. Papademetrio   +10 more
wiley   +1 more source

Beneficial Handling of Molecular Chaperones (Chaperonotherapy) in Glioblastoma and Neuroblastoma: Novel Therapeutic Targets or Potential Agents?

Cells
Molecular chaperones, especially Heat Shock Proteins (HSPs), play complex, context-dependent roles in cancer, particularly in nervous system (NS) tumors like glioblastoma (GBM) and neuroblastoma (NB).
Maria Antonella Augello, Nima Shadan, Giuseppa D’Amico, Rosario Barone, Celeste Caruso Bavisotto, Federica Scalia, Alessandra Maria Vitale   +6 more
doaj   +1 more source

Treatment of Fabry’s Disease with the Pharmacologic Chaperone Migalastat

New England Journal of Medicine, 2016
Fabry's disease, an X-linked disorder of lysosomal α-galactosidase deficiency, leads to substrate accumulation in multiple organs. Migalastat, an oral pharmacologic chaperone, stabilizes specific mutant forms of α-galactosidase, increasing enzyme trafficking to lysosomes.The initial assay of mutant α-galactosidase forms that we used to categorize 67 ...
Germain, Dominique P, Hughes, Derralynn A, Nicholls, Kathleen, Bichet, Daniel G, Giugliani, Roberto, Wilcox, William R, FELICIANI, Claudio, Shankar, Suma P, Ezgu, Fatih, Amartino, Hernan, Bratkovic, Drago, Feldt Rasmussen, Ulla, Nedd, Khan, Sharaf El Din, Usama, Lourenco, Charles M, Banikazemi, Maryam, Charrow, Joel, Dasouki, Majed, Finegold, David, Giraldo, Pilar, Goker Alpan, Ozlem, LONGO, NICOLA, Scott, C. Ronald, Torra, Roser, Tuffaha, Ahmad, Jovanovic, Ana, Waldek, Stephen, Packman, Seymour, Ludington, Elizabeth, Viereck, Christopher, Kirk, John, Yu, Julie, Benjamin, Elfrida R, Johnson, Franklin, Lockhart, David J, Skuban, Nina, Castelli, Jeff, Barth, Jay, Barlow, Carrolee, Schiffmann, Raphael   +39 more
openaire   +8 more sources

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

Diabetes Drug Discovery: hIAPP1–37 Polymorphic Amyloid Structures as Novel Therapeutic Targets

Molecules, 2018
Human islet amyloid peptide (hIAPP1–37) aggregation is an early step in Diabetes Mellitus. We aimed to evaluate a family of pharmaco-chaperones to act as modulators that provide dynamic interventions and the multi-target capacity (native state, cytotoxic
Isaac Fernández-Gómez, Marquiza Sablón-Carrazana, Alberto Bencomo-Martínez, Guadalupe Domínguez, Reyna Lara-Martínez, Nelly F. Altamirano-Bustamante, Luis Felipe Jiménez-García, Karina Pasten-Hidalgo, Rosa Angélica Castillo-Rodríguez, Perla Altamirano, Suchitil Rivera Marrero, Cristina Revilla-Monsalve, Peter Valdés-Sosa, Fabio Salamanca-Gómez, Eulalia Garrido-Magaña, Chryslaine Rodríguez-Tanty, Myriam M. Altamirano-Bustamante   +16 more
doaj   +1 more source

Pharmacological targeting of host chaperones protects from pertussis toxin in vitro and in vivo

Scientific Reports, 2021
Whooping cough is caused by Bordetella pertussis that releases pertussis toxin (PT) which comprises enzyme A-subunit PTS1 and binding/transport B-subunit.
Katharina Ernst, Ann-Katrin Mittler, Veronika Winkelmann, Carolin Kling, Nina Eberhardt, Anna Anastasia, Michael Sonnabend, Robin Lochbaum, Jan Wirsching, Moona Sakari, Arto T. Pulliainen, Ciaran Skerry, Nicholas H. Carbonetti, Manfred Frick, Holger Barth   +14 more
doaj   +1 more source

Role of misfolding in rare enzymatic deficits and use of pharmacological chaperones as therapeutic approach

Frontiers in Bioscience-Landmark, 2021
Cells have evolved sophisticated molecular control systems to maximize the efficiency of the folding process. However, any subtle alteration of the environment or the protein can lead to misfolding or affect the conformational plasticity of the native ...
Gioena Pampalone, Silvia Grottelli, Leonardo Gatticchi, Emilia Maria Lombardi, Ilaria Bellezza, Barbara Cellini   +5 more
doaj   +1 more source

Novel pharmacological chaperones that correct phenylketonuria in mice [PDF]

Human Molecular Genetics, 2012
Phenylketonuria (PKU) is caused by inherited phenylalanine-hydroxylase (PAH) deficiency and, in many genotypes, it is associated with protein misfolding. The natural cofactor of PAH, tetrahydrobiopterin (BH(4)), can act as a pharmacological chaperone (PC) that rescues enzyme function.
Sandra, Santos-Sierra, Johannes, Kirchmair, Anna M, Perna, Dunja, Reiss, Kristina, Kemter, Wulf, Röschinger, Hartmut, Glossmann, Søren W, Gersting, Ania C, Muntau, Gerhard, Wolber, Florian B, Lagler   +10 more
openaire   +2 more sources