Results 81 to 90 of about 57,493 (305)

Protein Folding and Mechanisms of Proteostasis [PDF]

International Journal of Molecular Sciences, 2015
Highly sophisticated mechanisms that modulate protein structure and function, which involve synthesis and degradation, have evolved to maintain cellular homeostasis. Perturbations in these mechanisms can lead to protein dysfunction as well as deleterious cell processes.
José Díaz-Villanueva, Raúl Díaz-Molina, Victor García-González   +2 more
openaire   +2 more sources

Lysosome‐Acidifying Nanoparticles Rescue A30P α‐Synuclein Induced Neuronal Death in Cellular and Drosophila Models of Parkinson's Disease

Advanced Healthcare Materials, EarlyView.
Lysosome‐targeted acidic nanoparticles based on a biodegradable poly(ethylene tetrafluorosuccinate‐co‐succinate) copolymer are engineered to restore impaired lysosomal acidification through pH‐responsive intracellular degradation. Localized acid release enhances autophagic proteolysis, reduces α‐synuclein accumulation, and preserves dopaminergic neuron
Chih Hung Lo, Mengda Ren, Gavin Wen Zhao Loi, Eka Norfaishanty Saipuljumri, Jonathan Indajang, Kah Leong Lim, Jialiu Zeng   +6 more
wiley   +1 more source

Transgenic sensors reveal compartment-specific effects of aggregation-prone proteins on subcellular proteostasis during aging

Cell Reports: Methods
Summary: Loss of proteostasis is a hallmark of aging that underlies many age-related diseases. Different cell compartments experience distinctive challenges in maintaining protein quality control, but how aging regulates subcellular proteostasis remains ...
Michelle Curley, Mamta Rai, Chia-Lung Chuang, Vishwajeeth Pagala, Anna Stephan, Zane Coleman, Maricela Robles-Murguia, Yong-Dong Wang, Junmin Peng, Fabio Demontis   +9 more
doaj   +1 more source

Proteomic analysis of cortical neuronal cultures treated with poly-arginine peptide-18 (R18) and exposed to glutamic acid excitotoxicity [PDF]

, 2019
Poly-arginine peptide-18 (R18) has recently emerged as a highly effective neuroprotective agent in experimental stroke models, and is particularly efficacious in protecting cortical neurons against glutamic acid excitotoxicity.
Anderton, Ryan S, Knuckey, Neville W, MacDougall, Gabriella, Mastaglia, Frank L, Meloni, Bruno P   +4 more
core   +1 more source

Restoration of CFTR function in patients with cystic fibrosis carrying the F508del-CFTR mutation [PDF]

, 2014
Restoration of BECN1/Beclin 1-dependent autophagy and depletion of SQSTM1/p62 by genetic manipulation or autophagy-stimulatory proteostasis regulators, such as cystamine, have positive effects on mouse models of human cystic fibrosis (CF). These measures
Amin R, Andrea Venerando, Angela Sepe, Anil Mehta, Anna Bossi, Anna Zolin, Antonella Tosco, Boyle MP BS, Carlo A Leone, Daniela De Stefano, Donaldson S., Fabiola De Gregorio, Fischer H, Gianni Bona, Gibson LE, Giuseppe De Rosa, Guido Kroemer, Illek B, Jayaraman S, Kerem B, Kerem Eithan HMO, Laura Salvadori, Legssyer R, Lorenzo A Pinna, Luigi Maiuri, Lukacs G, Maria C Maiuri, Massimo Pettoello-Mantovani, Masvidal L, Pharmaceuticals Cylene, Pharmaceuticals Cylene, Rosa Grassia, Speranza Esposito, Standardization of Spirometry, Stefano Guido, Stern M, Valeria R Villella, Valeria Raia, van Doorninck JH, Villella VR   +39 more
core   +4 more sources

Multisystem proteinopathy due to a homozygous p.Arg159His VCP mutation : a tale of the unexpected [PDF]

, 2020
ObjectiveTo assess the clinical, radiologic, myopathologic, and proteomic findings in a patient manifesting a multisystem proteinopathy due to a homozygous valosin-containing protein gene (VCP) mutation previously reported to be pathogenic in the ...
Azmi, A, Baets, J, Clemen, CS, De Bleecker, Jan, De Jonghe, P, De Ridder, W, Eichinger, L, Hofmann, A, Johnson, K, Maudsley, S, Schröder, R, Straub, V, Töpf, A   +12 more
core   +1 more source

Engineering of Glioblastoma‐Derived Biomimetic Vesicles and Their Structural and Molecular Features

Advanced Healthcare Materials, EarlyView.
ABSTRACT Biomimetic nanosystems and vesicles have arisen as a novel approach to design vesicular transport systems with diverse therapeutic potential. The ‘biomimetic’ strategy involves the integration of cell membrane components into lipid bilayers, conferring them with biological properties originating from the cell of origin. Until now, most studies
Noelia Hernández‐Lobato, Hanan Abumanhal‐Masarweh, Martí de Cabo, Pablo Guerra, Marilena Hadjidemetriou, Neus Lozano, Kostas Kostarelos   +6 more
wiley   +1 more source

Aging-induced proteostatic changes in the rat hippocampus identify ARP3, NEB2 and BRAG2 as a molecular circuitry for cognitive impairment.

PLoS ONE, 2013
Disturbed proteostasis as a particular phenotype of the aging organism has been advanced in C. elegans experiments and is also conceived to underlie neurodegenerative diseases in humans.
Philipp Ottis, Bianca Topic, Maarten Loos, Ka Wan Li, Angelica de Souza, Daniela Schulz, August B Smit, Joseph P Huston, Carsten Korth   +8 more
doaj   +1 more source

Integrating ER and Mitochondrial Proteostasis in the Healthy and Diseased Heart

Frontiers in Cardiovascular Medicine, 2020
The integrity of the proteome in cardiac myocytes is critical for robust heart function. Proteome integrity in all cells is managed by protein homeostasis or proteostasis, which encompasses processes that maintain the balance of protein synthesis ...
Adrian Arrieta, Erik A. Blackwood, Winston T. Stauffer, Christopher C. Glembotski   +3 more
doaj   +1 more source

Temporal requirements of SKN-1/NRF as a regulator of lifespan and proteostasis in Caenorhabditis elegans.

PLoS ONE, 2021
Lowering the activity of the Insulin/IGF-1 Signaling (IIS) cascade results in elevated stress resistance, enhanced protein homeostasis (proteostasis) and extended lifespan of worms, flies and mice. In the nematode Caenorhabditis elegans (C. elegans), the
Danielle Grushko, Hana Boocholez, Amir Levine, Ehud Cohen   +3 more
doaj   +1 more source