Zobrazeno 1 - 10
of 61
pro vyhledávání: '"Patricia Cmielewski"'
Autor:
Nicole Reyne, Ronan Smith, Patricia Cmielewski, Nina Eikelis, Mark Lawrence, Jennie Louise, Piraveen Pirakalathanan, David Parsons, Martin Donnelley
Publikováno v:
Scientific Reports, Vol 14, Iss 1, Pp 1-10 (2024)
Abstract Two cystic fibrosis (CF) rat models, one carrying the common Phe508del mutation and the other a nonsense cystic fibrosis transmembrane conductance regulator (CFTR) mutation (knockout) were previously characterised. Although relevant CFTR mRN
Externí odkaz:
https://doaj.org/article/f30e6698146645fa9909c5f556e1586c
Autor:
Nicole Reyne, Patricia Cmielewski, Alexandra McCarron, Ronan Smith, Elena Schneider-Futschik, Nina Eikelis, Piraveen Pirakalathanan, David Parsons, Martin Donnelley
Publikováno v:
Frontiers in Pharmacology, Vol 15 (2024)
Externí odkaz:
https://doaj.org/article/725af926fa9c429f9377c586aaa5b7a7
Autor:
Jiraporn Ousingsawat, Raquel Centeio, Nicole Reyne, Alexandra McCarron, Patricia Cmielewski, Rainer Schreiber, Gabriella diStefano, Dorothee Römermann, Ursula Seidler, Martin Donnelley, Karl Kunzelmann
Publikováno v:
Scientific Reports, Vol 14, Iss 1, Pp 1-12 (2024)
Abstract The Ca2+ activated Cl− channel TMEM16A (anoctamin 1; ANO1) is expressed in secretory epithelial cells of airways and intestine. Previous studies provided evidence for a role of ANO1 in mucus secretion. In the present study we investigated
Externí odkaz:
https://doaj.org/article/06c1135f56334127a708313a72426579
Autor:
Nicole Reyne, Patricia Cmielewski, Alexandra McCarron, Ronan Smith, Elena Schneider-Futschik, Nina Eikelis, Piraveen Pirakalathanan, David Parsons, Martin Donnelley
Publikováno v:
Frontiers in Pharmacology, Vol 15 (2024)
Introduction:Phe508del is the most common cystic fibrosis transmembrane conductance regulator (CFTR) gene variant that results in the recessive genetic disorder cystic fibrosis (CF). The recent development of highly effective CFTR modulator therapies
Externí odkaz:
https://doaj.org/article/d6e52064a46e407fbcacadcbcf9524f1
Autor:
Martin Donnelley, Patricia Cmielewski, Kaye Morgan, Juliette Delhove, Nicole Reyne, Alexandra McCarron, Nathan Rout-Pitt, Victoria Drysdale, Chantelle Carpentieri, Kathryn Spiers, Akihisa Takeuchi, Kentaro Uesugi, Naoto Yagi, David Parsons
Publikováno v:
Scientific Reports, Vol 12, Iss 1, Pp 1-14 (2022)
Abstract Gene vectors to treat cystic fibrosis lung disease should be targeted to the conducting airways, as peripheral lung transduction does not offer therapeutic benefit. Viral transduction efficiency is directly related to the vector residence ti
Externí odkaz:
https://doaj.org/article/8e5066133a14494fa29a90749fcb2c7c
To bead or not to bead: A review of Pseudomonas aeruginosa lung infection models for cystic fibrosis
Publikováno v:
Frontiers in Physiology, Vol 14 (2023)
Cystic fibrosis (CF) lung disease is characterised by recurring bacterial infections resulting in inflammation, lung damage and ultimately respiratory failure. Pseudomonas aeruginosa is considered one of the most important lung pathogens in those wit
Externí odkaz:
https://doaj.org/article/969e444d6b584b46a13f2b92e4981943
Single-Dose Lentiviral Mediated Gene Therapy Recovers CFTR Function in Cystic Fibrosis Knockout Rats
Autor:
Nicole Reyne, Patricia Cmielewski, Alexandra McCarron, Juliette Delhove, David Parsons, Martin Donnelley
Publikováno v:
Frontiers in Pharmacology, Vol 12 (2021)
Cystic fibrosis (CF) is a genetic disease caused by mutations in the CF transmembrane conductance regulator (CFTR) gene, resulting in defective ion transport in the airways. Addition of a functioning CFTR gene into affected airway cells has the poten
Externí odkaz:
https://doaj.org/article/76ef993215b24e49ac044df678348d35
Autor:
Alexandra McCarron, Nigel Farrow, Patricia Cmielewski, Emma Knight, Martin Donnelley, David Parsons
Publikováno v:
Frontiers in Pharmacology, Vol 12 (2021)
The lungs have evolved complex physical, biological and immunological defences to prevent foreign material from entering the airway epithelial cells. These mechanisms can also affect both viral and non-viral gene transfer agents, and significantly di
Externí odkaz:
https://doaj.org/article/5591e6bb406b45ad8d329865166be593
Autor:
Nigel Farrow, Patricia Cmielewski, Martin Donnelley, Nathan Rout-Pitt, Yuben Moodley, Ivan Bertoncello, David Parsons
Publikováno v:
Stem Cell Research & Therapy, Vol 9, Iss 1, Pp 1-8 (2018)
Abstract Background Airway disease is a primary cause of morbidity and early mortality for patients with cystic fibrosis (CF). Cell transplantation therapy has proven successful for treating immune disorders and may have the potential to correct the
Externí odkaz:
https://doaj.org/article/6e0a5ca029d24cec9546c61078b55b9b
Autor:
Victoria Drysdale, Patricia Cmielewski, Martin Donnelley, Nicole Reyne, David Parsons, Alexandra McCarron
Publikováno v:
Human Gene Therapy. 33:1062-1072
Natural airway defenses currently impede the efficacy of viral vector-mediated airway gene therapy. Conditioning airways before vector delivery can disrupt these barriers, improving viral vector access to target receptors and airway stem cells. This