Zobrazeno 1 - 10
of 208
pro vyhledávání: '"Christine E. Bear"'
Autor:
Henry Quach, Spencer Farrell, Ming Jia Michael Wu, Kayshani Kanagarajah, Joseph Wai-Hin Leung, Xiaoqiao Xu, Prajkta Kallurkar, Andrei L. Turinsky, Christine E. Bear, Felix Ratjen, Brian Kalish, Sidhartha Goyal, Theo J. Moraes, Amy P. Wong
Publikováno v:
Nature Communications, Vol 15, Iss 1, Pp 1-24 (2024)
Abstract Studying human fetal lungs can inform how developmental defects and disease states alter the function of the lungs. Here, we sequenced >150,000 single cells from 19 healthy human pseudoglandular fetal lung tissues ranging between gestational
Externí odkaz:
https://doaj.org/article/a4b68a6bade64f2b97f6c7af28a41953
Autor:
Adrian Constantin Tanjala, Jia Xin Jiang, Paul D. W. Eckford, Mohabir Ramjeesingh, Canhui Li, Ling Jun Huan, Gabrielle Langeveld, Claire Townsend, Daniel V. Paone, Jakob Busch-Petersen, Roman Pekhletski, LiPing Tang, Vamsee Raju, Steven M. Rowe, Christine E. Bear
Publikováno v:
Respiratory Research, Vol 25, Iss 1, Pp 1-10 (2024)
Abstract Background Cystic Fibrosis causing mutations in the gene CFTR, reduce the activity of the CFTR channel protein, and leads to mucus aggregation, airway obstruction and poor lung function. A role for CFTR in the pathogenesis of other muco-obst
Externí odkaz:
https://doaj.org/article/2da00d71ae6d4c6493740fb315b80270
Autor:
Sang Hyun Lim, Jamie Snider, Liron Birimberg‐Schwartz, Wan Ip, Joana C Serralha, Hugo M Botelho, Miquéias Lopes‐Pacheco, Madalena C Pinto, Mohamed Taha Moutaoufik, Mara Zilocchi, Onofrio Laselva, Mohsen Esmaeili, Max Kotlyar, Anna Lyakisheva, Priscilla Tang, Lucía López Vázquez, Indira Akula, Farzaneh Aboualizadeh, Victoria Wong, Ingrid Grozavu, Teuta Opacak‐Bernardi, Zhong Yao, Meg Mendoza, Mohan Babu, Igor Jurisica, Tanja Gonska, Christine E Bear, Margarida D Amaral, Igor Stagljar
Publikováno v:
Molecular Systems Biology, Vol 18, Iss 2, Pp 1-23 (2022)
Abstract Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) is a chloride and bicarbonate channel in secretory epithelia with a critical role in maintaining fluid homeostasis. Mutations in CFTR are associated with Cystic Fibrosis (CF), the mo
Externí odkaz:
https://doaj.org/article/2333634eb31448bfa35a483208141fd8
Autor:
Mina Ogawa, Jia-Xin Jiang, Sunny Xia, Donghe Yang, Avrilynn Ding, Onofrio Laselva, Marcela Hernandez, Changyi Cui, Yuichiro Higuchi, Hiroshi Suemizu, Craig Dorrell, Markus Grompe, Christine E. Bear, Shinichiro Ogawa
Publikováno v:
Nature Communications, Vol 12, Iss 1, Pp 1-19 (2021)
Current protocols to generate cholangiocytes from human pluripotent cells produce immature cells. Here the authors identify retinoic acid, BMP, cAMP and Rho kinase pathways as regulators of cholangiocyte maturation, and generate ciliated cholangiocyt
Externí odkaz:
https://doaj.org/article/e2c37e554f8c4d829b4039f62abf625f
A Cell-Based Optimised Approach for Rapid and Efficient Gene Editing of Human Pluripotent Stem Cells
Autor:
Sara Cuevas-Ocaña, Jin Ye Yang, Magomet Aushev, George Schlossmacher, Christine E. Bear, Nicholas R. F. Hannan, Neil D. Perkins, Janet Rossant, Amy P. Wong, Michael A. Gray
Publikováno v:
International Journal of Molecular Sciences, Vol 24, Iss 12, p 10266 (2023)
Introducing or correcting disease-causing mutations through genome editing in human pluripotent stem cells (hPSCs) followed by tissue-specific differentiation provide sustainable models of multiorgan diseases, such as cystic fibrosis (CF). However, l
Externí odkaz:
https://doaj.org/article/531cdd449add4766881e623a2c423d1d
Autor:
Onofrio Laselva, Evgeniy V. Petrotchenko, C. Michael Hamilton, Zafar Qureshi, Christoph H. Borchers, Robert N. Young, Christine E. Bear
Publikováno v:
STAR Protocols, Vol 3, Iss 2, Pp 101258- (2022)
Summary: We describe a protocol to identify the binding site(s) for a drug called ivacaftor that potentiates the CFTR chloride channel. We use photoaffinity probes—based on the structure of ivacaftor—to covalently modify the CFTR protein at the r
Externí odkaz:
https://doaj.org/article/d780e2dbabf949ef8626b66fff79d3bc
Autor:
Tarini N. A. Gunawardena, Zoltán Bozóky, Claire Bartlett, Hong Ouyang, Paul D. W. Eckford, Theo J. Moraes, Felix Ratjen, Tanja Gonska, Christine E. Bear
Publikováno v:
Cells, Vol 12, Iss 8, p 1174 (2023)
It has been suggested that in vitro studies of the rescue effect of CFTR modulator drugs in nasal epithelial cultures derived from people with cystic fibrosis have the potential to predict clinical responses to the same drugs. Hence, there is an inte
Externí odkaz:
https://doaj.org/article/7828993941e14c3486c8bc71a324d365
Autor:
Steven Erwood, Onofrio Laselva, Teija M.I. Bily, Reid A. Brewer, Alexandra H. Rutherford, Christine E. Bear, Evgueni A. Ivakine
Publikováno v:
Molecular Therapy: Methods & Clinical Development, Vol 17, Iss , Pp 1118-1128 (2020)
Nonsense-mediated decay (NMD) is a major pathogenic mechanism underlying a diversity of genetic disorders. Nonsense variants tend to lead to more severe disease phenotypes and are often difficult targets for small molecule therapeutic development as
Externí odkaz:
https://doaj.org/article/eb2405ef414a48d59db42b474b399baf
Publikováno v:
EBioMedicine, Vol 73, Iss , Pp 103660- (2021)
Recent strides towards precision medicine in Cystic Fibrosis (CF) have been made possible by patient-derived in-vitro assays with the potential to predict clinical response to small molecule-based therapies. Here, we discuss the status of primary and
Externí odkaz:
https://doaj.org/article/19a94a5005ec4e2cadbf87feaf0e7d46
Autor:
Onofrio Laselva, Zafar Qureshi, Zhi-Wei Zeng, Evgeniy V. Petrotchenko, Mohabir Ramjeesingh, C. Michael Hamilton, Ling-Jun Huan, Christoph H. Borchers, Régis Pomès, Robert Young, Christine E. Bear
Publikováno v:
iScience, Vol 24, Iss 6, Pp 102542- (2021)
Summary: Ivacaftor (VX-770) was the first cystic fibrosis transmembrane conductance regulator (CFTR) modulatory drug approved for the treatment of patients with cystic fibrosis. Electron cryomicroscopy (cryo-EM) studies of detergent-solubilized CFTR
Externí odkaz:
https://doaj.org/article/8c79a33405ed438787e6e0dfeeeb1030