Laura Schaupp

Bridging the airway microbiome and targeted therapy in bronchiectasis: multi-omics insights, endotypes and emerging therapies

Christina S. Thornton, Laura Schaupp, Michael M. Tunney, Marcus Alexander Mall

Bronchiectasis is a heterogeneous chronic airway disease primarily driven by persistent infection, microbial dysbiosis and dysregulated host immunity. While culture-based microbiology has historically informed clinical management, advances in high-throughput sequencing and multi-omic technologies have transformed our understanding of the airway ecosystem, revealing …

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Systemic effects of cystic fibrosis transmembrane conductance regulator modulators on the plasma and serum proteome

Kerstin Fentker, Marieluise Kirchner, Matthias Ziehm, Sylvia Niquet et autres

Cystic fibrosis (CF) is caused by dysfunction of the cystic fibrosis transmembrane conductance regulator (CFTR) anion channel in epithelial organs leading to a complex multi-organ disease. CFTR modulator drugs improve mutant CFTR function and markedly improve clinical outcomes in people with CF …

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Preventive intrapulmonary treatment with Ligilactobacillus murinus reduces airway inflammation and mucus plugging in mice with cystic fibrosis-like lung disease

Robert Brock, Laura Schaupp, A.H. Schutte, Zhe Zhou-Suckow et autres

Background Chronic airway dysbiosis plays an important role in the pathogenesis of cystic fibrosis (CF) lung disease and may serve as a therapeutic target. However, studies investigating the effects of direct therapeutic targeting of the airway microbiome are lacking. In this study, …

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Lung Function Changes in Response to Antibiotic Therapy for Pulmonary Exacerbations in Primary Ciliary Dyskinesia

Ruth Maria Urbantat, B R Fuhlrott, Hannah Nussstein, Ana Stamatova-Tomova et autres

Background: Primary ciliary dyskinesia (PCD) is a rare disease caused by mutations in genes important for ciliary function and assembly. Ciliary dysfunction leads to an impaired mucociliary clearance, resulting in chronic progressive muco-obstructive lung disease. First causative treatments are underway, but clinical …

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Genetic Deletion of Muc5b Reduces Interstitial Lung Disease in Neonatal Nedd4-2 Deficient Mice

Nathalie Smyczek, Julie C. Meyer Auf Der Heyde, DH W Leitz, Laura Schaupp et autres

MUC5B has been implicated as a major risk factor for the development of pulmonary fibrosis. However, insights into its role in the in vivo pathogenesis of interstitial lung disease (ILD) remain incomplete. We previously developed a mouse model ( Nedd4-2 -/- ) …

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Changes in Sputum Viscoelastic Properties and Airway Inflammation in Primary Ciliary Dyskinesia are Comparable to Cystic Fibrosis on Elexacaftor/Tezacaftor/Ivacaftor Therapy

Ruth Maria Urbantat, Hannah Nussstein, Kerstin Fentker, A Loewe et autres

Background: Primary ciliary dyskinesia (PCD) and cystic fibrosis (CF) are muco-obstructive lung diseases that are both caused by genetically determined defects in mucociliary clearance, which triggers a cascade that evolves into a ‘vicious vortex’ of airway mucus plugging, chronic polymicrobial infection and …

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192 Sputum viscoelastic properties and airway inflammation in cystic fibrosis on elexacaftor/tezacaftor/ivacaftor therapy are comparable to primary ciliary dyskinesia

Laura Schaupp, Hannah Nussstein, Ruth Maria Urbantat, Kerstin Fentker et autres

Changes in sputum viscoelastic properties and airway inflammation in primary ciliary dyskinesia are comparable to cystic fibrosis on elexacaftor/tezacaftor/ivacaftor therapy

Hannah Nussstein, Ruth Maria Urbantat, Kerstin Fentker, Julia Duerr et autres

BACKGROUND: Primary ciliary dyskinesia (PCD) and cystic fibrosis (CF) are muco-obstructive lung diseases that are caused by distinct genetically determined defects in mucociliary clearance; however, knowledge on the relative severity of airway mucus dysfunction and chronic inflammation remains limited. The aim of …

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WS08.06Integrative computational analysis of longitudinal effects of elexacaftor/tezacaftor/ivacaftor on sputum microbiome and proteome in patients with cystic fibrosis

Laura Schaupp, Rebecca Luise Knoll, Kerstin Fentker, J. Nazat Martinez Medina et autres

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Genetic Deletion of Muc5b Reduces Interstitial Lung Disease in Neonatal Nedd4-2 Deficient Mice

Nathalie Smyczek, Julie C. Meyer Auf Der Heyde, Dominik H. W. Leitz, Laura Schaupp et autres

MUC5B has been implicated as a major risk factor for the development of idiopathic pulmonary fibrosis. However, insights into its role in the in vivo pathogenesis of interstitial lung disease (ILD) remain limited. We previously developed a mouse model for early onset …

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Operating and Biocontainment Procedures of a Facility for Laboratory Mice with a Natural Microbiome: Immunophenotyping Procedure

Natascha Drude, Kai Diederich, Claudia U. Duerr, Nadine Haase et autres

The use of laboratory mice with a natural microbiome, such as "Wildling mice", offers a promising research tool for both basic and applied science due to their close resemblance to the human superorganism. However, the breeding and maintenance of these mice, which …

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Systemic effects of cystic fibrosis transmembrane conductance regulator (CFTR) modulators on the blood proteome

Kerstin Fentker, Marieluise Kirchner, Matthias Ziehm, Sylvia Niquet et autres

Summary Cystic fibrosis (CF), resulting from a dysfunction in the cystic fibrosis transmembrane conductance regulator (CFTR), affects multiple organs through mucus obstruction and differences in secretion. The CFTR modulator drug combination elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA, ETI) has markedly improved clinical symptoms, but its broader …

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