Search Results - "Graeber, Simon Y."

CRISPR-Based Adenine Editors Correct Nonsense Mutations in a Cystic Fibrosis Organoid Biobank by Geurts, Maarten H., de Poel, Eyleen, Amatngalim, Gimano D., Oka, Rurika, Meijers, Fleur M., Kruisselbrink, Evelien, van Mourik, Peter, Berkers, Gitte, de Winter-de Groot, Karin M., Michel, Sabine, Muilwijk, Danya, Aalbers, Bente L., Mullenders, Jasper, Boj, Sylvia F., Suen, Sylvia W.F., Brunsveld, Jesse E., Janssens, Hettie M., Mall, Marcus A., Graeber, Simon Y., van Boxtel, Ruben, van der Ent, Cornelis K., Beekman, Jeffrey M., Clevers, Hans

“…Adenine base editing (ABE) enables enzymatic conversion from A-T into G-C base pairs. ABE holds promise for clinical application, as it does not depend on the…”
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Comparison of microbiomes from different niches of upper and lower airways in children and adolescents with cystic fibrosis by Boutin, Sébastien, Graeber, Simon Y, Weitnauer, Michael, Panitz, Jessica, Stahl, Mirjam, Clausznitzer, Diana, Kaderali, Lars, Einarsson, Gisli, Tunney, Michael M, Elborn, J Stuart, Mall, Marcus A, Dalpke, Alexander H

“…Changes in the airway microbiome may be important in the pathophysiology of chronic lung disease in patients with cystic fibrosis. However, little is known…”
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A novel thiol-saccharide mucolytic for the treatment of muco-obstructive lung diseases by Addante, Annalisa, Raymond, Wilfred, Gitlin, Irina, Charbit, Annabelle, Orain, Xavier, Scheffler, Aaron Wolfe, Kuppe, Aditi, Duerr, Julia, Daniltchenko, Maria, Drescher, Marika, Graeber, Simon Y, Healy, Anne-Marie, Oscarson, Stefan, Fahy, John V, Mall, Marcus A

“…Mucin disulfide cross-links mediate pathologic mucus formation in muco-obstructive lung diseases. MUC-031, a novel thiol-modified carbohydrate compound,…”
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Ion mobility-tandem mass spectrometry of mucin-type O-glycans by Bechtella, Leïla, Chunsheng, Jin, Fentker, Kerstin, Ertürk, Güney R., Safferthal, Marc, Polewski, Łukasz, Götze, Michael, Graeber, Simon Y., Vos, Gaël M., Struwe, Weston B., Mall, Marcus A., Mertins, Philipp, Karlsson, Niclas G., Pagel, Kevin

“…The dense O -glycosylation of mucins plays an important role in the defensive properties of the mucus hydrogel. Aberrant glycosylation is often correlated with…”
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Personalized CFTR Modulator Therapy for G85E and N1303K Homozygous Patients with Cystic Fibrosis by Graeber, Simon Y, Balázs, Anita, Ziegahn, Niklas, Rubil, Tihomir, Vitzthum, Constanze, Piehler, Linus, Drescher, Marika, Seidel, Kathrin, Rohrbach, Alexander, Röhmel, Jobst, Thee, Stephanie, Duerr, Julia, Mall, Marcus A, Stahl, Mirjam

“…CFTR modulator therapy with elexacaftor/tezacaftor/ivacaftor (ETI) has been approved for people with CF and at least one F508del allele in Europe. In the US,…”
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Effects of elexacaftor/tezacaftor/ivacaftor therapy on mental health of patients with cystic fibrosis by Piehler, Linus, Thalemann, Ralf, Lehmann, Christine, Thee, Stephanie, Röhmel, Jobst, Syunyaeva, Zulfiya, Stahl, Mirjam, Mall, Marcus A, Graeber, Simon Y

“…The CFTR modulator drug elexacaftor/tezacaftor/ivacaftor (ETI) was shown to improve CFTR function and clinical symptoms in patients with cystic fibrosis (CF)…”
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Changes in Microbiome Dominance Are Associated With Declining Lung Function and Fluctuating Inflammation in People With Cystic Fibrosis by Frey, Dario L, Bridson, Calum, Dittrich, Susanne, Graeber, Simon Y, Stahl, Mirjam, Wege, Sabine, Herth, Felix, Sommerburg, Olaf, Schultz, Carsten, Dalpke, Alexander, Mall, Marcus A, Boutin, Sébastien

“…Airway inflammation and microbiome dysbiosis are hallmarks of cystic fibrosis ( ) lung disease. However, longitudinal studies are needed to decipher which…”
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Effects of lumacaftor-ivacaftor therapy on cystic fibrosis transmembrane conductance regulator function in F508del homozygous patients with cystic fibrosis aged 2-11 years by Berges, Julian, Graeber, Simon Y, Hämmerling, Susanne, Yu, Yin, Krümpelmann, Arne, Stahl, Mirjam, Hirtz, Stephanie, Scheuermann, Heike, Mall, Marcus A, Sommerburg, Olaf

“…Lumacaftor/ivacaftor was approved for the treatment of patients with cystic fibrosis who are homozygous for F508del aged 2 years and older following positive…”
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Long-term effects of lumacaftor/ivacaftor on paranasal sinus abnormalities in children with cystic fibrosis detected with magnetic resonance imaging by Wucherpfennig, Lena, Wuennemann, Felix, Eichinger, Monika, Seitz, Angelika, Baumann, Ingo, Stahl, Mirjam, Graeber, Simon Y, Zhao, Shengkai, Chung, Jaehi, Schenk, Jens-Peter, Alrajab, Abdulsattar, Kauczor, Hans-Ulrich, Mall, Marcus A, Sommerburg, Olaf, Wielpütz, Mark O

“…Chronic rhinosinusitis (CRS) usually presents with nasal congestion, rhinorrhea and anosmia impacts quality of life in cystic fibrosis (CF). Especially…”
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Comparison of Oropharyngeal Microbiota from Children with Asthma and Cystic Fibrosis by Dalpke, Alexander H., Mall, Marcus, Legatzki, Antje, Dittrich, Susanne A., Graeber, Simon Y., Stahl, Mirjam, Depner, Martin, Boutin, Sébastien, von Mutius, Erika

“…A genuine microbiota resides in the lungs which emanates from the colonization by the oropharyngeal microbiota. Changes in the oropharyngeal microbiota might…”
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A Volatile and Dynamic Longitudinal Microbiome Is Associated With Less Reduction in Lung Function in Adolescents With Cystic Fibrosis by Metzger, Marisa I, Graeber, Simon Y, Stahl, Mirjam, Sommerburg, Olaf, Mall, Marcus A, Dalpke, Alexander H, Boutin, Sébastien

“…Progressive impairment in lung function caused by chronic polymicrobial airway infection remains the major cause of death in patients with cystic fibrosis…”
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The future of cystic fibrosis treatment: from disease mechanisms to novel therapeutic approaches by Graeber, Simon Y, Mall, Marcus A

“…With the 2019 breakthrough in the development of highly effective modulator therapy providing unprecedented clinical benefits for over 90% of patients with…”
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Magnetic resonance imaging detects onset and association with lung disease severity of bronchial artery dilatation in cystic fibrosis by Leutz-Schmidt, Patricia, Optazaite, Daiva-Elzbieta, Sommerburg, Olaf, Eichinger, Monika, Wege, Sabine, Steinke, Eva, Graeber, Simon Y, Puderbach, Michael U, Schenk, Jens-Peter, Alrajab, Abdulsattar, Triphan, Simon M F, Kauczor, Hans-Ulrich, Stahl, Mirjam, Mall, Marcus A, Wielpütz, Mark O

“…Bronchial artery dilatation (BAD) is associated with haemoptysis in advanced cystic fibrosis (CF) lung disease. Our aim was to evaluate BAD onset and its…”
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TRACK-CF prospective cohort study: Understanding early cystic fibrosis lung disease by Steinke, Eva, Sommerburg, Olaf, Graeber, Simon Y, Joachim, Cornelia, Labitzke, Christiane, Nissen, Gyde, Ricklefs, Isabell, Rudolf, Isa, Kopp, Matthias V, Dittrich, Anna-Maria, Mall, Marcus A, Stahl, Mirjam

“…Lung disease as major cause for morbidity in patients with cystic fibrosis (CF) starts early in life. Its large phenotypic heterogeneity is partially explained…”
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Comparison of Lung Clearance Index and Magnetic Resonance Imaging for Assessment of Lung Disease in Children with Cystic Fibrosis by Stahl, Mirjam, Wielpütz, Mark O, Graeber, Simon Y, Joachim, Cornelia, Sommerburg, Olaf, Kauczor, Hans-Ulrich, Puderbach, Michael, Eichinger, Monika, Mall, Marcus A

“…Early onset and progression of lung disease in children with cystic fibrosis (CF) indicates that sensitive noninvasive outcome measures are needed for…”
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Effects of Elexacaftor/Tezacaftor/Ivacaftor Therapy on CFTR Function in Patients with Cystic Fibrosis and One or Two F508del Alleles by Graeber, Simon Y, Vitzthum, Constanze, Pallenberg, Sophia T, Naehrlich, Lutz, Stahl, Mirjam, Rohrbach, Alexander, Drescher, Marika, Minso, Rebecca, Ringshausen, Felix C, Rueckes-Nilges, Claudia, Klajda, Jan, Berges, Julian, Yu, Yin, Scheuermann, Heike, Hirtz, Stephanie, Sommerburg, Olaf, Dittrich, Anna-Maria, Tümmler, Burkhard, Mall, Marcus A

“…The CFTR (cystic fibrosis transmembrane conductance regulator) modulator combination elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) was shown to improve…”
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Depression Symptoms in Patients with Cystic Fibrosis Fluctuate at Baseline and Improve with Elexacaftor/Tezacaftor/Ivacaftor Therapy by Piehler, Linus, Thalemann, Ralf, Lehmann, Christine, Stahl, Mirjam, Mall, Marcus A, Graeber, Simon Y

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Preventive Inhalation of Hypertonic Saline in Infants with Cystic Fibrosis (PRESIS). A Randomized, Double-Blind, Controlled Study by Stahl, Mirjam, Wielpütz, Mark O, Ricklefs, Isabell, Dopfer, Christian, Barth, Sandra, Schlegtendal, Anne, Graeber, Simon Y, Sommerburg, Olaf, Diekmann, Gesa, Hüsing, Johannes, Koerner-Rettberg, Cordula, Nährlich, Lutz, Dittrich, Anna-Maria, Kopp, Matthias V, Mall, Marcus A

“…Cystic fibrosis (CF) lung disease starts in early infancy, suggesting that preventive treatment may be most beneficial. Lung clearance index (LCI) and chest…”
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Effects of Lumacaftor-Ivacaftor Therapy on Cystic Fibrosis Transmembrane Conductance Regulator Function in Phe508del Homozygous Patients with Cystic Fibrosis by Graeber, Simon Y, Dopfer, Christian, Naehrlich, Lutz, Gyulumyan, Lena, Scheuermann, Heike, Hirtz, Stephanie, Wege, Sabine, Mairbäurl, Heimo, Dorda, Marie, Hyde, Rebecca, Bagheri-Hanson, Azadeh, Rueckes-Nilges, Claudia, Fischer, Sebastian, Mall, Marcus A, Tümmler, Burkhard

“…The combination of the CFTR (cystic fibrosis transmembrane conductance regulator) corrector lumacaftor with the potentiator ivacaftor has been approved for the…”
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Magnetic Resonance Imaging Detects Progression of Lung Disease and Impact of Newborn Screening in Preschool Children with Cystic Fibrosis by Stahl, Mirjam, Steinke, Eva, Graeber, Simon Y, Joachim, Cornelia, Seitz, Christoph, Kauczor, Hans-Ulrich, Eichinger, Monika, Hämmerling, Susanne, Sommerburg, Olaf, Wielpütz, Mark O, Mall, Marcus A

“…Previous cross-sectional studies have demonstrated that chest magnetic resonance imaging (MRI) is sensitive to detect early lung disease in infants and…”
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