Phenotypic profiling of CFTR modulators in patient-derived respiratory epithelia

Autor: Saumel Ahmadi, Zoltan Bozoky, Michelle Di Paola, Sunny Xia, Canhui Li, Amy P. Wong, Leigh Wellhauser, Steven V. Molinski, Wan Ip, Hong Ouyang, Julie Avolio, Julie D. Forman-Kay, Felix Ratjen, Jeremy A. Hirota, Johanna Rommens, Janet Rossant, Tanja Gonska, Theo J. Moraes, Christine E. Bear

Publikováno v: npj Genomic Medicine, Vol 2, Iss 1, Pp 1-10 (2017)

Cystic fibrosis: toward personalized therapies A new method for evaluating drug responses in patient-derived respiratory tissue promises to help determine the best treatment for each patient with cystic fibrosis (CF). CF patients are highly susceptib

Externí odkaz: https://doaj.org/article/7ad6ed4d84964bd982f178ae435366d1

Palmitate‐mediated induction of neuropeptide Y expression occurs through intracellular metabolites and not direct exposure to proinflammatory cytokines

Autor: Leigh Wellhauser, Richard P. Bazinet, Denise D. Belsham, Wenyuan He, Kathyrn E Hopperton, Jim T. C. Chen, Andy Tran

Publikováno v: Journal of Neurochemistry. 159:574-589

A contributing factor to the development of obesity is the consumption of a diet high in saturated fatty acids, such as palmitate. These fats induce hypothalamic neuroinflammation, which dysregulates neuronal function and induces orexigenic neuropept

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::e10bd355c75a8cc9a0d79f46b7758523
https://doi.org/10.1111/jnc.15504

ORKAMBI-Mediated Rescue of Mucociliary Clearance in Cystic Fibrosis Primary Respiratory Cultures Is Enhanced by Arginine Uptake, Arginase Inhibition, and Promotion of Nitric Oxide Signaling to the Cystic Fibrosis Transmembrane Conductance Regulator Channel

Autor: Sunny Xia, Theo J. Moraes, Claire Bartlett, Janet Jiang, Leigh Wellhauser, Tanja Gonska, Yu-Sheng Wu, Hong Ouyang, Onofrio Laselva, Alexandria Lew, Wan Ip, Christine E. Bear, Saumel Ahmadi

Publikováno v: Molecular Pharmacology. 96:515-525

ORKAMBI, a combination of the corrector, lumacaftor, and the potentiator, ivacaftor, partially rescues the defective processing and anion channel activity conferred by the major cystic fibrosis-causing mutation, F508del, in in vitro studies. Clinical

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::5243a55b077b06eb706b6c9c65c40c1e
https://doi.org/10.1124/mol.119.117143

Orkambi® and amplifier co‐therapy improves function from a rare CFTR mutation in gene‐edited cells and patient tissue

Autor: J.P. Miller, Ling Jun Huan, Saumel Ahmadi, Felix Ratjen, Régis Pomès, Paul D. W. Eckford, Hong Ouyang, Leigh Wellhauser, Christine E. Bear, Adriana Villella, Ellen Li, Theo J. Moraes, Kethika Kulleperuma, Peter N. Ray, Michelle Di Paola, Onofrio Laselva, Steven Molinski, Wan Ip, Po-Shun Lee, Tanja Gonska, Kai Du

Publikováno v: EMBO Molecular Medicine

The combination therapy of lumacaftor and ivacaftor (Orkambi ® ) is approved for patients bearing the major cystic fibrosis (CF) mutation: ΔF508 . It has been predicted that Orkambi ® could treat patients with rarer mutations of similar “theraty

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::2aaadb2a10accd16a9627ce93c3d4764
http://europepmc.org/articles/PMC5582412

High fat induces acute and chronic inflammation in the hypothalamus: effect of high-fat diet, palmitate and TNF-α on appetite-regulating NPY neurons

Autor: Jennifer A. Chalmers, M B Wheeler, Leigh Wellhauser, Denise D. Belsham, Serge Luquet, Prasad S. Dalvi, D Q Tran, D-Yd Han, Julien Castel, Ying Liu, V Luo

Publikováno v: International Journal of Obesity
International Journal of Obesity, Nature Publishing Group, 2017, 41 (1), pp.149-158. ⟨10.1038/ijo.2016.183⟩

International audience; BACKGROUND: Consumption of dietary fat is one of the key factors leading to obesity. High-fat diet (HFD)-induced obesity is characterized by induction of inflammation in the hypothalamus; however, the temporal regulation of pr

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::f98804b94231c08ab30937d73161a2fa
https://doi.org/10.1038/ijo.2016.183