Results 121 to 130 of about 12,522 (293)

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

The proteomic differences and expression of fatty acid‐binding protein 6 (FABP6) associated with gastrointestinal injury in horses with oral administration of a clinical dose of phenylbutazone

Equine Veterinary Journal, EarlyView.
Abstract Background Phenylbutazone (PBZ) can potentially induce gastrointestinal ulceration, and early detection of PBZ‐induced gastroenteropathy will be useful for the diagnosis, treatment, and prevention of PBZ toxicity. Objectives To identify putative proteins associated with equine gastric ulcer syndrome after clinical dose (4.4 mg/kg ...
Ruethaiwan Vinijkumthorn, Nawarus Prapaiwan, Thanapon Chotikaprakal, Phirom Prompiram, Narumon Phaonakrop, Sittiruk Roytrakul, Parichart Tesena   +6 more
wiley   +1 more source

Characterizing CFTR modulated sweat chloride response across the cf population: Initial results from the CHEC-SC study [PDF]

, 2022
Nicole Mayer-Hamblett, Edith T. Zemanick, Katherine Odem‐Davis, Donald R. VanDevanter, Mark N. Warden, Steven M. Rowe, J. Young, MW Konstan, for-the-CHEC-SC-Study-Group   +8 more
openalex   +1 more source

Lung organoids as a human system for Mycobacteria infection modeling and drug testing

The FEBS Journal, EarlyView.
Mycobacterial infections, including tuberculosis (TB) and infections by nontuberculous mycobacteria (NTM), are still public health issues. In 2023, TB caused 1.25 million deaths, while NTM remain a clinical challenge for patients with cystic fibrosis (CF).
Stephen Adonai Leon‐Icaza, Romain Vergé, Raoul Mazars, Laurence Berry, Céline Cougoule   +4 more
wiley   +1 more source

Heat-stable enterotoxins of enterotoxigenic Escherichia coli and their impact on host immunity [PDF]

, 2019
Enterotoxigenic Escherichia coli (ETEC) are an important diarrhea-causing pathogen and are regarded as a global threat for humans and farm animals. ETEC possess several virulence factors to infect its host, including colonization factors and enterotoxins.
Cox, Eric, Devriendt, Bert, Luo, Yu, Wang, Haixiu, Zhong, Zifu   +4 more
core   +1 more source

EPS8.05 One-minute sit-to-stand test improves with CFTR modulators [PDF]

, 2023
M. Penelle, Anne-Sophie Aubriot, C. R. Gonçalves, Christophe Goubau, Silvia Berardis, Grégory Reychler, Sophie Gohy   +6 more
openalex   +1 more source

Targeting a genetic defect: cystic fibrosis transmembrane conductance regulator modulators in cystic fibrosis [PDF]

European Respiratory Review, 2013
Cystic fibrosis (CF) is caused by genetic mutations that affect the cystic fibrosis transmembrane conductance regulator (CFTR) protein. These mutations can impact the synthesis and transfer of the CFTR protein to the apical membrane of epithelial cells ...
Nico Derichs
doaj  

Krisanaklan Reduces Intestinal Anion and Fluid Secretion Through Inhibition of Na+/K+‐ATPase and K+ Channel Activity

Annals of the New York Academy of Sciences, EarlyView.
Krisanaklan reduces CFTR‐dependent intestinal chloride and fluid secretion by inhibiting the Na+,K+‐ATPase and K+ channels in epithelial cells. Consequently, this natural, plant‐derived product may limit secretory diarrhea caused by a diverse array of microbial pathogens. However, by dissipating the transmembrane Na+ gradient, Krisanaklan also inhibits
Tessa A. Groeneweg, Erdene Baigal, Anny Leung, Gert‐Jan Kremers, Marcel J. C. Bijvelds   +4 more
wiley   +1 more source

P039 The clinical impact of triple CFTR modulator therapy in a cohort of people with cystic fibrosis and severe lung disease

, 2021
O. Fanthorpe, S. Caskey, D.G. Downey
openalex   +1 more source

Distinct Roles of SLC26A3 and CFTR in Surface pH Regulation and Bicarbonate Secretion in Human Intestinal Epithelium

Acta Physiologica, Volume 242, Issue 2, February 2026.
ABSTRACT Background and Aims Colonic bicarbonate secretion is mediated by the chloride/bicarbonate exchanger SLC26A3 and the cystic fibrosis transmembrane conductance regulator (CFTR). Dysfunction of either causes luminal acidosis, altered mucus properties, and inflammation.
Mahdi Amiri, Azam Salari, Ursula Seidler
wiley   +1 more source