A5046588195- Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
- Peptidase Inhibition and Analysis
- Toxin Mechanisms and Immunotoxins
- Occupational exposure and asthma
- Cellular Mechanics and Interactions
- Electrospun Nanofibers in Biomedical Applications
- Occupational and environmental lung diseases
- Chronic Myeloid Leukemia Treatments
- Bone health and treatments
- Cell Adhesion Molecules Research
- Pulmonary Hypertension Research and Treatments
- Tissue Engineering and Regenerative Medicine
- Cancer-related gene regulation
- Connective tissue disorders research
- Signaling Pathways in Disease
Ludwig-Maximilians-Universität München
2018-2022
Helmholtz Zentrum München
2015-2022
German Center for Lung Research
2018-2022
Comprehensive Urology
2017
Idiopathic pulmonary fibrosis (IPF) is characterized by excessive deposition of extracellular matrix, in particular, collagens. Two IPF therapeutics, nintedanib and pirfenidone, decelerate lung function decline, but their underlying mechanisms action are poorly understood. In this study, we sought to analyze effects on collagen synthesis maturation at important regulatory levels. Primary human fibroblasts from patients with healthy donors were treated (0.01-1.0 μM) or pirfenidone (100-1,000...
Rationale: Increased abundance and stiffness of the extracellular matrix, in particular collagens, is a hallmark idiopathic pulmonary fibrosis (IPF). FK506-binding protein 10 (FKBP10) collagen chaperone, mutations which have been indicated reduction matrix (e.g., osteogenesis imperfecta).Objectives: To assess expression function FKBP10 IPF.Methods: We assessed bleomycin-induced lung (using quantitative reverse transcriptase–polymerase chain reaction, Western blot, immunofluorescence),...
Lung fibrosis is characterized by excessive deposition of extracellular matrix (ECM), in particular collagens, fibroblasts the interstitium. Transforming growth factor-β1 (TGF-β1) alters expression many (ECM) components produced fibroblasts, but such changes ECM composition as well modulation collagen post-translational modification (PTM) levels have not been comprehensively investigated. Here, we performed mass spectrometry (MS)-based proteomics analyses to assess deposited cultured lung...
Antibodies are central effectors of the adaptive immune response, widespread used therapeutics, but also potentially disease-causing biomolecules. Antibody folding catalysts in plasma cell incompletely defined. Idiopathic pulmonary fibrosis (IPF) is a fatal chronic lung disease with increasingly recognized autoimmune features. We found elevated expression FK506-binding protein 11 (FKBP11) IPF lungs where FKBP11 specifically localized to antibody-producing cells. Suggesting general role...
In idiopathic pulmonary fibrosis (IPF), fibroblasts gain a more migratory phenotype and excessively secrete extracellular matrix (ECM), ultimately leading to alveolar scarring progressive dyspnea. Here, we analyzed the effects of deficiency FK506-binding protein 10 (FKBP10), potential IPF drug target, on primary human lung fibroblast (phLF) adhesion migration. Using siRNA, FKBP10 expression was inhibited in phLF absence or presence 2ng/ml transforming growth factor-β1 (TGF-β1) 0.1mM...
<b>Rationale:</b> Idiopathic pulmonary fibrosis (IPF) is an irreversible lung disease characterized by excessive deposition of collagen. The two IPF therapeutics nintedanib and pirfenidone decelerate progression, but the underlying mechanisms are poorly understood. This study comprehensively analyzes effects both drugs on collagen synthesis at several regulatory levels. <b>Methods:</b> Primary human fibroblasts were treated with (0.01-1.0µM) or (0.1-1.0mM) without TGF-ß1. Effects fibrotic...
<b>Background:</b> Idiopathic pulmonary fibrosis (IPF) is a fatal chronic lung disease characterized by fibroblast activation and accumulation of collagen other extracellular matrix (ECM) components. We have previously proposed the chaperone FK506-binding protein 10 (FKBP10) as potential therapeutic target for IPF. The genes encoding FKBP10 prolyl-3-hydroxylase 4 (P3H4), novel component ER-resident folding complex in skin bone, are controlled common bidirectional promoter. Regulation...
<b>Rationale:</b> In idiopathic pulmonary fibrosis, fibroblasts gain a more migratory phenotype and secrete excessive amounts of extracellular matrix (ECM), ultimately leading to alveolar scarring loss lung function. We have recently identified FK506-binding protein 10 (FKBP10) as an important regulator collagen synthesis secretion in IPF. Here, we analyzed effects FKBP10 deficiency on primary human (phLF) adhesion migration. <b>Methods:</b> Using siRNA, expression was downregulated phLF...
We have recently identified the chaperone and peptidyl-prolyl cis-trans isomerase FK506-binding protein 10 (FKBP10) as a profibrotic mediator in idiopathic pulmonary fibrosis (IPF), fatal lung disease with increasingly evident autoimmune features. In this study, we sought to assess expression, localization, regulation function of related protein, FKBP11, fibrosis. FKBP11 was highly increased IPF specifically expressed by tissue-resident plasma cells both lungs lymphoid organs. B cell...
We have recently identified the chaperone and peptidyl-prolyl isomerase FK506-binding protein 10 (FKBP10) as a profibrotic mediator in idiopathic pulmonary fibrosis (IPF). In this study, we sought to assess expression, localization, regulation function of related protein, FKBP11, lung fibrosis.
Idiopathic pulmonary fibrosis (IPF) is characterized by fibroblast activation and accumulation of collagen in the alveolar space. We have previously proposed chaperone FK506-binding protein 10 (FKBP10) as a therapeutic target for IPF. Expression <i>FKBP10</i> prolyl-3-hydroxylase 4 (<i>P3H4</i>), novel component ER-resident folding complex, are controlled common bidirectional promoter, but regulation function P3H4 lung unknown. Transcriptomic proteomic analysis was performed using lungs from...