bioRxiv Science⌕ Search

Biology subjects

Geillinger-Kaestle, K.

Publications and source records attributed to Geillinger-Kaestle, K..

3 recordsLinked to original sources

AXL-GAS6/PROS1 Interaction: A Critical Switch Between Aberrant- and Healthy Repair Following Alveolar Lung Injury

RationaleIdiopathic pulmonary fibrosis (IPF) is a progressive lung disease characterized by aberrant alveolar repair and excessive fibrosis. The TAM-family receptor tyrosine kinase AXL, activated by GAS6 and PROS1, is implicated in tissue remodeling, but ligand-specific AXL signaling during alveolar repair remains poorly defined. ObjectivesTo investigate ligand specific AXL signaling in IPF and how it impacts epithelial proliferation and repair after alveolar injury in-vivo and in-vitro. MethodsSingle cell RNA sequencing was utilized to understand cell specific expression patterns in IPF patients, followed by functional studies in primary human cell culture and functional spatial digital profiling (FuncOmap) analysis in patient tissue. Longitudinal assessment of repair process after alveolar-specific injury in-vivo was used to complement the in-vitro approach. ResultsAXL expression showed enrichment in basal and aberrant basaloid cells of IPF patients. In-vitro GAS6 increased proliferation of basal cells, while PROS1 counteracted this effect. FuncOmap analysis demonstrates direct in-situ interactions between AXL and both ligands, providing evidence for biological relevance. Investigating longitudinal repair processes in-vivo revealed dynamic regulation of AXL ligands as well as AXL. ConclusionsThese findings highlight the importance of ligand-specific AXL signaling in lung repair and suggest that it dysregulation may contribute to IPF pathogenesis, offering potential therapeutic targets for restoring normal repair processes.

molecular biology↗

In Silico Treatment: a computational framework for animal model selection and drug assessment

The translation of findings from animal models to human disease is a fundamental part in the field of drug development. However, only a small proportion of promising preclinical results in animals translate to human pathophysiology. This underscores the necessity for novel data analysis strategies to accurately evaluate the most suitable animal model for a specific purpose, ensuring cross-species translatability. To address this need, we present In Silico Treatment (IST), a computational method to assess translation of disease-related molecular expression patterns between animal models and humans. By simulating changes observed in animals onto humans, IST provides a holistic picture of how well animal models recapitulate key aspects of human disease, or how treatments transform pathogenic expression patterns to healthy ones. Furthermore, IST highlights particular genes that influence molecular features of pathogenesis or drug mode of action. We demonstrate the potential of IST with three applications using bulk transcriptomics data. First, we assessed two mouse models for idiopathic pulmonary fibrosis (IPF): one involving injury with intra-tubular Bleomycin exposure, and the other Adeno-associated-virus-induced, TGF{beta}1-mediated tissue transformation (AAV6.2-TGF{beta}1). Both models exhibited gene expression patterns resembling extracellular matrix derangement in human IPF, whereas differences in VEGF-driven vascularization were observed. Second, we confirmed known features of non-alcoholic steatohepatitis (NASH) mouse models, including choline-deficient, l-amino acid-defined diet (CDAA), carbon tetrachloride hepatotoxicity injury (CCl4) and bile duct ligation surgery (BDL). Overall, the three mouse models recapitulated expression changes related to fibrosis in human NASH, whereas model-specific differences were found in lipid metabolism, inflammation, and apoptosis. Third, we reproduced the strong anti-fibrotic signature and induction of the PPAR signaling observed in the Elafibranor experimental treatment for NASH in the CDAA model. We validated the contribution of known disease-related genes to the findings made with IST in the IPF and NASH applications. The complete data integration IST framework, including an interactive app to integrate and compare datasets, is made available as an open-source R package. Author summaryPreclinical testing plays a pivotal role in the drug development process, serving as a crucial evaluation phase before a new drug can be tested on humans in clinical trials. The drug must undergo a rigorous evaluation in in vivo and in vitro preclinical studies to assess its safety and efficacy. However, positive outcomes in preclinical animal models do not always translate to positive results in humans, mainly due to biological differences. Therefore, selecting an animal model that closely mirrors human disease traits and detecting and accounting for model limitations is of paramount importance. Over the last decade, the availability of gene expression data in both animals and humans has substantially increased. Gene expression states and perturbations are routinely employed as a proxy to predict and understand changes in disease states. Here, we developed In Silico Treatment, a computational method designed to overlay the gene expression changes observed in animals onto humans, quantifying the change in human disease status. We applied this method to mouse models for idiopathic pulmonary fibrosis and non-alcoholic steatohepatitis, two severe fibrotic diseases. We successfully identified known features of the disease models and provide a granular gene-level rationale behind our predictions. Consequently, our method shows promise as an effective approach to improve animal model selection and thus clinical translation.

bioinformatics↗

Characterization of a flexible AAV-DTR/DT mouse model of acute epithelial lung injury

Background & aimRecurring epithelial injury and aberrant repair are considered as a major driver of idiopathic pulmonary fibrosis (IPF) leading to chronic inflammation, fibroblast activation and ultimately to scarring and stiffening of the lung. As decline of lung function is the first reported symptom by IPF patients and occurs once fibrosis is firmly established, animal models are required to study early disease-driving mechanisms. MethodsWe developed a novel and flexible mouse model of acute epithelial injury based on adeno-associated virus (AAV) variant 6.2 mediated expression of the human diphtheria toxin receptor (DTR). Following intratracheal administration of diphtheria toxin (DT), a cell-specific death of bronchial epithelial and alveolar epithelial type II cells can be observed. ResultsDetailed characterization of the AAV-DTR/DT mouse model revealed increasing cell numbers in bronchoalveolar lavage (BAL; macrophages, neutrophils, and atypical cells) and elevation of apoptotic cells and infiltrated leukocytes in lung tissue, which were dependent of viral genome load and DT dose. Cytokine levels in BAL fluid showed different patterns dependent of viral genome load with IFN{gamma}, TNF, and IP-10 increasing and IL-5 and IL-6 decreasing, while lung function was not affected. Additionally, laser-capture microdissection-based proteomics of bronchial and alveolar epithelium showed upregulated immune and inflammatory response in all epithelial cell regions and extracellular matrix deposition in infiltrated alveoli, while proteins involved in pulmonary surfactant synthesis, alveolar fluid clearance and alveolar-capillary barrier were downregulated in the parenchyma. ConclusionOur novel AAV-DTR/DT model resembles specific aspects of pulmonary diseases like IPF and acute respiratory distress syndrome. Short summary for social mediaA novel and flexible mouse model of acute epithelial lung injury based on AAV-mediated expression of the human diphtheria toxin receptor followed by intratracheal instillation of diphtheria toxin resembles specific aspects of pulmonary diseases like IPF.

cell biology↗