Results 111 to 120 of about 57,493 (305)

Remodeling the Proteostasis Network to Rescue Glucocerebrosidase Variants by Inhibiting ER-Associated Degradation and Enhancing ER Folding [PDF]

, 2013
Gaucher’s disease (GD) is characterized by loss of lysosomal glucocerebrosidase (GC) activity. Mutations in the gene encoding GC destabilize the protein’s native folding leading to ER-associated degradation (ERAD) of the misfolded enzyme.
AH Futerman, Andrea Dardis, AR Sawkar, AR Sawkar, AR Sawkar, C Dionisi-Vici, C Ficicioglu, D Pelled, D Ron, DJ Termine, DS Ong, DS Wishart, E Beutler, E Fiebiger, E Korkotian, E Lloyd-Evans, EF Eckenrode, F Wang, F Wang, F Wang, Fan Wang, FL Bygrave, GA Grabowski, J Lu, JE Chipuk, JE Wraith, JH Lin, JH Lin, KS Hruska, Laura Segatori, M Kitamura, M Schmitz, M Schroder, ME Grace, MJ Berridge, PJ Meikle, PM Hoogerbrugge, Q Wang, R Khanna, RJ Youle, S Gunther, S Gupta, S Oyadomari, SZ Xu, UH Schueler, YP Rong, Z Yu   +46 more
core   +3 more sources

T Cell Exhaustion in Cancer Immunotherapy: Heterogeneity, Mechanisms, and Therapeutic Opportunities

Advanced Science, EarlyView.
T cell exhaustion limits immunotherapy efficacy. This article delineates its progression from stem‐like to terminally exhausted states, governed by persistent antigen, transcription factors, epigenetics, and metabolism. It maps the exhaustion landscape in the TME and proposes integrated reversal strategies, providing a translational roadmap to overcome
Yang Yu, Xiaoyu Yao, Qingyang Wang, Mengrui Yang, Runze Li, Jiangjiang Qin, Jing Zhuang, Changgang Sun   +7 more
wiley   +1 more source

Adapt, Recycle, and Move on: Proteostasis and Trafficking Mechanisms in Melanoma [PDF]

, 2016
Melanoma has emerged as a paradigm of a highly aggressive and plastic cancer, capable to co-opt the tumour stroma in order to adapt to the hostile microenvironment, suppress immunosurveillance mechanisms and disseminate.
Hannelore Maes, Patrizia Agostinis, Seyma Demirsoy, Shaun Martin   +3 more
core   +2 more sources

Cis‐ and Trans‐Regulatory Factors Independently Shape Phenotypic Heterogeneity of Retinitis Pigmentosa

Advanced Science, EarlyView.
A zebrafish model carrying an identical human RHO S334X allele reveals two independent genetic layers shaping retinitis pigmentosa (RP) severity: a protective 3‐bp cis‐regulatory insertion that attenuates transgene expression, and a dominant trans‐acting modifier that restores a severe phenotype.
Cong Cui, Kotone Nakagawa, Takumi Tateno, Ayaka Dan, Dexin Meng, Yoshihiro Omori, Soma Tomihara, Suzuri Okamoto, Shigeru Sato, Motokazu Tsujikawa   +9 more
wiley   +1 more source

TFEB regulates lysosomal proteostasis [PDF]

, 2013
Loss-of-function diseases are often caused by destabilizing mutations that lead to protein misfolding and degradation. Modulating the innate protein homeostasis (proteostasis) capacity may lead to rescue of native folding of the mutated variants, thereby
Abrahamov, Alberto di Ronza, Altarescu, Ashley Ake, Balch, Balch, Beutler, Brown, Cho, Christophe, Desnick, Dionisi-Vici, Fan Wang, Ferrao-Gonzales, Fu, Futerman, Gennarino, Gieselmann, Ginzburg, Grabowski, Grabowski, Grace, Hruska, Huang, Karageorgos, Kim, Kiselyov, Koprivica, Krivit, Krivit, Krivit, Laura Segatori, Lieberman, Lopes, Lu, Lu, Lubke, Ma, Maley, Marco Sardiello, Marzia Savini, Medina, Meikle, Michelakakis, Mu, Mu, Neufeld, Nigg, Ong, Palmieri, Parr, Powers, Reczek, Rohrbach, Ron, Roversi, Sands, Sardiello, Sardiello, Sawkar, Sawkar, Schmitz, Schueler, Seregin, Settembre, Shapiro, Steingrimsson, Subramanian, Tropak, Tsuji, Tsuji, Tsunemi, Vandesompele, Wang, Wang, Wang, Wei, Welch, Wensi Song, Yu, Zhao   +80 more
core   +1 more source

Plant Genetic Engineering: Technological Pathways, Application Scenarios, and Future Directions

Advanced Science, EarlyView.
This review maps the fast‐evolving landscape of plant genetic engineering, linking enabling platforms with trait‐focused applications in architecture optimization, stress resilience, yield improvement, and quality enhancement. It highlights how genome editing, transgenic strategies, and emerging multi‐gene approaches reshape breeding pipelines, while ...
Peilin Wang, Wenzhi Wang, Dongjiao Wang, Qibin Wu, Youxiong Que   +4 more
wiley   +1 more source

Impairment of lipid homeostasis causes lysosomal accumulation of endogenous protein aggregates through ESCRT disruption

eLife
Protein aggregation increases during aging and is a pathological hallmark of many age-related diseases. Protein homeostasis (proteostasis) depends on a core network of factors directly influencing protein production, folding, trafficking, and degradation.
John Yong, Jacqueline E Villalta, Ngoc Vu, Matthew A Kukurugya, Niclas Olsson, Magdalena Preciado López, Julia R Lazzari-Dean, Kayley Hake, Fiona E McAllister, Bryson D Bennett, Calvin H Jan   +10 more
doaj   +1 more source

Proteostasis and Diseases of the Motor Unit

Frontiers in Molecular Neuroscience, 2016
The accumulation in neurons of aberrant protein species, the pathological hallmark of many neurodegenerative diseases, results from a global impairment of key cellular processes governing protein synthesis/degradation and repair mechanisms, also known as the proteostasis network (PN).
Rinaldi, C, Mäger, I, Wood, M
openaire   +3 more sources

Mitochondrial Enzymes Mimetic Ultrasmall Palladium Nanozymes Prevent Senescence and Neurodegeneration Through Metabolic Reprogramming

Advanced Science, EarlyView.
Simply constructed polyvinylpyrrolidone (PVP) modified palladium nanoparticles (PdP NPs) as cytochrome c oxidase (CcO) and superoxide dismutase (SOD) mimic are promising multifunctional nanoreactors to boost mitochondrial respiration chain function and refine homeostasis via regulation of mitochondrial dynamics and proteostasis sensor UPRmt, which ...
Wenshu Cong, Haiming Jing, Zinan Li, Wenjing Zhang, Nan Zhang, Yingqiu Xie, Shan Gao, Yuanyu Huang, Junyu Ning   +8 more
wiley   +1 more source

DNA‐Induced Entropic Gain Triggers an Allosteric Switch for Biomolecular Condensation of Heat Shock Transcription Factor 1

Angewandte Chemie, EarlyView.
Hsf1 undergoes phase separation around target DNA, activating stress‐responsive transcription. DNA binding tunes conformational dynamics of Hsf1DBD, which propagates through large‐scale domain rearrangements involving DBD–IDR interactions to regulate phase separation. This hierarchical framework illustrates how local structural events at the side chain
Soichiro Kawagoe, Hiroyuki Kumeta, Tomohide Saio   +2 more
wiley   +2 more sources