Results 51 to 60 of about 858,069 (214)

CFTR trafficking mutations disrupt cotranslational protein folding by targeting biosynthetic intermediates

Nature Communications, 2020
Cystic fibrosis (CF) is a lethal genetic disease that is primarily caused by misfolding of the cystic fibrosis transmembrane conductance regulator (CFTR). Here authors show that disease-causing mutations located within the first nucleotide binding domain
Hideki Shishido, Jae Seok Yoon, Zhongying Yang, William R. Skach   +3 more
doaj   +1 more source

F127-SE-tLAP thermosensitive hydrogel alleviates bleomycin-induced skin fibrosis via TGF-β/Smad pathway

Molecular Medicine
Background Skin fibrosis affects the normal function of the skin. TGF-β1 is a key cytokine that affects organ fibrosis. The latency-associated peptide (LAP) is essential for TGF-β1 activation.
Zhiqin Cao, Keke Zhang, Jingruo Liu, Yu Pan, Jiayi Shi, Luxin Li, Xiaocan Sun, Shiqi Li, Xiaohuan Yuan, Dan Wu   +9 more
doaj   +1 more source

Impact of Achromobacter xylosoxidans isolation on the respiratory function of adult patients with cystic fibrosis

ERJ Open Research, 2019
Background The prevalence of Achromobacter xylosoxidans lung isolation in cystic fibrosis (CF) patients has increased, but the impact on lung function is controversial. The aim of this study was to evaluate the long-term effects of A.
Macha Tetart, Frederic Wallet, Maeva Kyheng, Sylvie Leroy, Thierry Perez, Olivier Le Rouzic, Benoit Wallaert, Anne Prevotat   +7 more
doaj   +1 more source

A non-randomised single centre cohort study, comparing standard and modified bowel preparations, in adults with cystic fibrosis requiring colonoscopy

BMC Gastroenterology, 2019
Background Adults with cystic fibrosis (CF) have been reported to be at five to ten-fold risk (25 to 30 fold risk after solid organ transplant) of colorectal cancer (CRC) than the general population.
A. G. Matson, J. P. Bunting, A. Kaul, D. J. Smith, J. Stonestreet, K. Herd, R. S. Hodgson, S. C. Bell   +7 more
doaj   +1 more source

Clinical implications of interstitial pneumonia with autoimmune features diagnostic criteria in idiopathic pulmonary fibrosis: A case control study [PDF]

, 2023
Sara Tomassetti, Claudia Ravaglia, Silvia Puglisi, Athol U. Wells, Jay H. Ryu, Marcello Bosi, Alessandra Dubini, Sara Piciucchi, Francesco Girelli, Paola Parronchi, Federico Lavorini, Elisabetta Rosi, Valentina Luzzi, Marco Matucci‐Cerinic, Venerino Poletti   +14 more
openalex   +1 more source

Cystic fibrosis and the role of the physiotherapist

South African Journal of Physiotherapy, 1981
Cystic fibrosis is an inherited disease, the major problems being pancreatic malabsorption and progressive respiratory involvement, although numerous other organs may also be involved.
Anne Miot, J. M. Pettifor, I. Reef
doaj   +1 more source

Increased fat mass and obesity risk after elexacaftor–tezacaftor–ivacaftor therapy in young adults with cystic fibrosis

Frontiers in Nutrition
BackgroundWhen people with cystic fibrosis (PwCFs) are treated with cystic fibrosis transmembrane conductance regulator protein modulator (CFTRm), it leads to changes in body composition.
Ana Merino Sánchez-Cañete, Concepción Marina López Cárdenes, Saioa Vicente Santamaría, José Ramón Gutiérrez Martínez, Marta Suárez González, María Álvarez Merino, David González Jiménez   +6 more
doaj   +1 more source

Can TERT rs2853669 polymorphysm indicate fibrosis in sarcoidosis? [PDF]

, 2023
Sümeyye Kement, Eda Turgut Uğurtay, Aslı Tanrıvermiş Sayit, Pınar Tascı, Meftun Ünsal   +4 more
openalex   +1 more source

A smartphone app for preschool wheezing and reliability of medical history collection

Italian Journal of Pediatrics
Background The use of mobile applications helps improving self-management in adolescents with asthma. However, no evidence is available for children with preschool wheezing.
Nicola Ullmann, Adriana Fracchiolla, Alessandra Boni, Valentina Negro, Federica Porcaro, Antonio Di Marco, Salvatore Tripodi, Renato Cutrera   +7 more
doaj   +1 more source

Lung function improvement on triple modulators: high-resolution, nationwide data from the Danish Cystic Fibrosis Cohort

ERJ Open Research
Background People living with cystic fibrosis in Denmark had early, universal access to triple modulator treatment with elexacaftor/tezacaftor/ivacaftor.
Christian Leo-Hansen, Daniel Faurholt-Jepsen, Tavs Qvist, Christine Højte, Bibi U. Nielsen, Thomas Bryrup, Esben H. Henriksen, Terese Katzenstein, Marianne Skov, Inger H.M. Mathiesen, Majbritt Jeppesen, Søren Jensen-Fangel, Hanne V. Olesen, Frederik Fouirnaies Buchvald, Kim Gjerum Nielsen, Espen Jimenez-Solem, Christian Ritz, Tacjana Pressler, Mette F. Olsen, the TransformCF Study Group   +19 more
doaj   +1 more source