Results 91 to 100 of about 1,208,918 (256)

Mechanical Cues Regulate Cargo Sorting and Export at the Golgi

Advanced Science, EarlyView.
A pathway is identified linking extracellular matrix stiffness to secretion via Src–FAK–AMPK–GBF1 signaling. Golgi‐localized GBF1 acts as a mechanotransducer whose phosphorylation controls post‐Golgi cargo sorting toward secretion or lysosomal degradation.
Greta Serafino, Stefania Forciniti, Edoardo Scarpa, Antonio Ranieri, Lucia Santorelli, Gabriella De Blasi, Sara Costantini, Eunjoo Jun Lee, Antonio Galeone, Alessia Calcagnì, Marinella Pirozzi, Loretta L. del Mercato, Rossella Venditti, Giovanni D'Angelo, Seetharaman Parashuraman, Tiziano Verri, Giuseppe Gigli, Elizabeth S. Sztul, Paolo Grumati, Loris Rizzello, Domenico Russo, Riccardo Rizzo   +21 more
wiley   +1 more source

CFTR activity and mitochondrial function [PDF]

, 2013
Cystic Fibrosis (CF) is a frequent and lethal autosomal recessive disease, caused by mutations in the gene encoding the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR).
Santa Coloma, Tomás Antonio, Valdivieso, Ángel Gabriel   +1 more
core   +1 more source

Selective PAR2 Inhibition Attenuates HDM‐Induced Th1/Th2 Responses in Human Epithelial and Murine Models of Allergic Rhinitis and Asthma

International Forum of Allergy &Rhinology, EarlyView.
ABSTRACT Background Allergic rhinitis (AR) and asthma are involved in complex interactions between Th1 and Th2 inflammatory pathways. House dust mite (HDM) activates protease‐activated receptor 2 (PAR2) to trigger inflammatory responses, but current treatments often provide inadequate control.
Miran Kang, Yohan Seo, Ju Hee Seo, Yeonsu Jeong, Hyejin Jeon, Su‐Myeong Jang, Chang‐Hoon Kim, Wan Namkung, Hyung‐Ju Cho   +8 more
wiley   +1 more source

miRNA-221 is elevated in cystic fibrosis airway epithelial cells and regulates expression of ATF6. [PDF]

, 2015
Background MicroRNA (miRNA) and messenger RNA (mRNA) expression differs in cystic fibrosis (CF) versus non-CF bronchial epithelium. Here, the role of miRNA in basal regulation of the transcription factor ATF6 was investigated in bronchial epithelial ...
Agrawal, Raman, Greene, Catherine M, Mall, Marcus A, McElvaney, Noel G, Oglesby, Irene K   +4 more
core   +2 more sources

Resilience to Endoplasmic Reticulum Stress Mitigates Membrane Hyperexcitability Underlying Late Disease Onset in a Murine Model of SCA6

Annals of Neurology, EarlyView.
Objective An enduring puzzle in many inherited neurological disorders is the late onset of symptoms despite expression of function‐impairing mutant protein early in life. We examined the basis for onset of impairment in spinocerebellar ataxia type 6 (SCA6), a canonical late‐onset neurodegenerative ataxia which results from a polyglutamine expansion in ...
Haoran Huang, Taylor L. Charron, Min Fu, Miranda Dunn, Deborah M. Jones, Praveen Kumar, Satoshi Ishishita, Allan‐Hermann Pool, Ashwinikumar Kulkarni, Genevieve Konopka, Vikram G. Shakkottai   +10 more
wiley   +1 more source

Biology of Cholangiocytes: From Bench to Bedside [PDF]

, 2016
Cholangiocytes, the lining epithelial cells in bile ducts, are an important subset of liver cells. They are activated by endogenous and exogenous stimuli and are involved in the modification of bile volume and composition.
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core   +1 more source

Optimization of pharmaceutical research and development by early‐phase assessment of investigational medicinal products

British Journal of Clinical Pharmacology, EarlyView.
Small and mid‐sized pharmaceutical innovators often have limited in‐house health economics and market access expertise, and may struggle to align development strategies of investigational medicinal products with health system needs and payer expectations.
Zoltán Kaló, Attila Imre, Gábor Varga, Sándor Kovács, György Tímár, Balázs Nagy   +5 more
wiley   +1 more source

Clinical development of triple-combination CFTR modulators for cystic fibrosis patients with one or two F508del alleles

ERJ Open Research, 2019
Cystic fibrosis (CF) is caused by mutations in the CF transmembrane conductance regulator gene (CFTR) that result in diminished quantity and/or function of the CFTR anion channel.
Jennifer L. Taylor-Cousar, Marcus A. Mall, Bonnie W. Ramsey, Edward F. McKone, Elizabeth Tullis, Gautham Marigowda, Charlotte M. McKee, David Waltz, Samuel M. Moskowitz, Jessica Savage, Fengjuan Xuan, Steven M. Rowe   +11 more
doaj   +1 more source

The role of modulators in cystic fibrosis related diabetes

Journal of Clinical & Translational Endocrinology, 2022
The development and introduction of modulator therapies have completely shifted the paradigm for the treatment of cystic fibrosis (CF). Highly effective modulator therapies have driven marked improvements in lung function, exacerbation rate, weight and ...
Lina Merjaneh, Sana Hasan, Nader Kasim, Katie Larson Ode   +3 more
doaj   +1 more source

Quantitative Systems Toxicology Predicts Ivacaftor‐Induced Oxidative Stress Contributes to CFTR Modulator Hepatotoxicity

Clinical Pharmacology &Therapeutics, EarlyView.
Cystic fibrosis (CF) is a chronic hereditary disease that affects tens of thousands of people worldwide. The introduction of CFTR modulator therapies such as elexacaftor/tezacaftor/ivacaftor (ETI) has significantly improved the quality of life of people with CF.
Alan Shi, Cole Cornwell, Kyunghee Yang, Paul M. Beringer   +3 more
wiley   +1 more source