Results 121 to 130 of about 71,270 (242)

Roles of ER Membrane Protein Complex in Protein Biogenesis and Quality Control in the Lung and Beyond

Cell Proliferation, EarlyView.
The endoplasmic reticulum membrane protein complex (EMC) is an evolutionarily conserved, multi‐subunit transmembrane protein complex crucial to membrane protein biogenesis and cellular protein quality control. This review systematically examines the structure, functions and disease‐associated regulatory mechanisms of EMC across multiple organ systems ...
Yan Qiao, Yuqing Sun, Yutao Huang, Qinghang Meng, Xinhua Lin, Wei Wei, Xiaofang Tang   +6 more
wiley   +1 more source

A Powerful and Universal Preimplantation Genetic Diagnosis Protocol for Cystic Fibrosis

European Medical Journal Reproductive Health, 2018
Background: Cystic fibrosis (CF) is one of the most common indications of preimplantation genetic diagnosis (PGD) for monogenic disorders worldwide. Aims: The aim of this article was to report a universal and powerful assay easily applicable to all ...
Victoria Viart, Aliya Ishmukhametova, Stéphanie Plaza, Garance Verrière, Florielle Saguet, Mireille Claustres, Anne Girardet   +6 more
doaj  

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

IRBIT a Master Regulator of Cell Physiology [PDF]

, 2017
(excerpt) Hormones and neurotransmitters regulate cell functions by binding to their receptors, which activate intracellular signaling and produce the physiological response [1].
Bouyer, Patrice G, Cook, Kelli, Petreska, Natasa   +2 more
core   +1 more source

Murine and human CFTR exhibit different sensitivities to CFTR potentiators

American Journal of Physiology-Lung Cellular and Molecular Physiology, 2015
Development of therapeutic molecules with clinical efficacy as modulators of defective CFTR includes efforts to identify potentiators that can overcome or repair the gating defect in mutant CFTR channels. This has taken a great leap forward with the identification of the potentiator VX-770, now available to patients as “Kalydeco.” Other small molecules
Guiying, Cui, Nael A, McCarty
openaire   +3 more sources

Transcriptomic profiling of gill biopsies to define predictive markers for seawater survival in farmed Atlantic salmon

Journal of Fish Biology, EarlyView.
Abstract Wild Atlantic salmon migrate to sea following completion of a developmental process known as parr‐smolt transformation (PST), which establishes a seawater (SW) tolerant phenotype. Effective imitation of this aspect of anadromous life history is a crucial aspect of commercial salmon production, with current industry practice being marred by ...
Lars Grønvold, Mattis J. van Dalum, Anja Striberny, Domniki Manousi, Trine Ytrestøyl, Turid Mørkøre, Solomon Boison, Bjarne Gjerde, Even Jørgensen, Simen R. Sandve, David G. Hazlerigg   +10 more
wiley   +1 more source

Correction of CFTR function in nasal epithelial cells from cystic fibrosis patients predicts improvement of respiratory function by CFTR modulators

Scientific Reports, 2017
Clinical studies with modulators of the Cystic Fibrosis Transmembrane conductance Regulator (CFTR) protein have demonstrated that functional restoration of the mutated CFTR can lead to substantial clinical benefit.
Iwona M. Pranke, Aurélie Hatton, Juliette Simonin, Jean Philippe Jais, Françoise Le Pimpec-Barthes, Ania Carsin, Pierre Bonnette, Michael Fayon, Nathalie Stremler-Le Bel, Dominique Grenet, Matthieu Thumerel, Julie Mazenq, Valerie Urbach, Myriam Mesbahi, Emanuelle Girodon-Boulandet, Alexandre Hinzpeter, Aleksander Edelman, Isabelle Sermet-Gaudelus   +17 more
doaj   +1 more source

Expression of corticoid‐regulatory genes in the gills of Atlantic salmon (Salmo salar) parr and smolt and during salinity acclimation

Journal of Fish Biology, EarlyView.
Abstract In teleost fishes, cortisol is the major corticoid and has both glucocorticoid and mineralocorticoid actions. However, how fish tissues discriminate between these distinct corticosteroid actions is unclear. In mammals, the major factors responsible for intracellular corticosteroid regulation are glucocorticoid receptors (grs) and the ...
Makoto Kusakabe, Takashi Yada, Graham Young, Amy M. Regish, Stephen D. McCormick   +4 more
wiley   +1 more source

Cystic fibrosis therapy: from symptoms to the cause of the disease

Вавиловский журнал генетики и селекции
Cystic fibrosis (CF) is a disease with a broad clinical and genetic spectrum of manifestations, significantly impacting the quality and duration of life of patients.
T N. Kireeva, D. I. Zhigalina, N. A. Skryabin   +2 more
doaj   +1 more source

Genetic aspects of digestive diseases. Part 1

Терапевтический архив, 2010
The paper presents the data available in the literature on mutations in known genes in pancreatitis, such as cationic trypsinogen (PRSS1), pancreatic secretory trypsin inhibitor (PSTI/SPINK1), cystic fibrosis (CFTR), and apolipoprotein E (APOE) genes, as
Irina Nikolaevna Grigor'eva, Tat'yana Mikhaylovna Nikitenko, Aleksandr Vilenovich Tikhonov, Tat'yana Ivanovna Romanova, V N Maksimov, Elena Vladimirovna Shakhtshneyder, Sof'ya Konstantinovna Malyutina, Mikhail Ivanovich Voevoda, I N Grigoryeva, T M Nikitenko, A V Tikhonov, T I Romanova, V N Maksimov, E V Shakhtshneider, S K Malyutina, M I Voyevoda   +15 more
doaj