A5064244090- Genetic and Kidney Cyst Diseases
- Genetic Syndromes and Imprinting
- Renal and related cancers
- Hedgehog Signaling Pathway Studies
- Biotin and Related Studies
- Cancer Genomics and Diagnostics
- Computational Drug Discovery Methods
- T-cell and B-cell Immunology
- Ocular Disorders and Treatments
- Kruppel-like factors research
- Hippo pathway signaling and YAP/TAZ
- Click Chemistry and Applications
- CAR-T cell therapy research
- Immune Cell Function and Interaction
- Polyamine Metabolism and Applications
- Erythrocyte Function and Pathophysiology
Saarland University
2024-2025
Czech Academy of Sciences, Institute of Molecular Genetics
2020-2024
Charles University
2023-2024
Abstract The kinase LCK and CD4/CD8 co-receptors are crucial components of the T cell antigen receptor (TCR) signaling machinery, leading to key fate decisions. Despite decades research, roles CD4–LCK CD8–LCK interactions in TCR triggering vivo remain unknown. In this study, we created animal models expressing endogenous levels modified resolve whether how co-receptor-bound drives signaling. We demonstrated that role depends on co-receptor which it is bound. CD8-bound largely dispensable for...
Ascorbate peroxidase (APEX) is a versatile labeling enzyme used for live-cell proteomics at high spatial and temporal resolution. However, toxicity of its substrate hydrogen peroxide background by endogenous peroxidases limit use to in vitro studies specific cell types. By combining APEX2 with D-amino acid oxidase locally produce peroxide, we establish more versatile, improved APEX (iAPEX) workflow that minimizes reduces non-specific labeling. We employ iAPEX perform cellular microdomain,...
Bardet–Biedl syndrome (BBS) is a pleiotropic ciliopathy caused by dysfunction of primary cilia. More than half BBS patients carry mutations in one eight genes encoding for subunits protein complex, the BBSome, which mediates trafficking ciliary cargoes. In this study, we elucidated mechanisms BBSome assembly living cells and how process spatially regulated. We generated large library human cell lines deficient particular subunit expressing another tagged with fluorescent protein. analyzed...
Bardet-Biedl syndrome (BBS) is an archetypal ciliopathy caused by dysfunction of primary cilia. BBS affects multiple tissues, including the kidney, eye and hypothalamic satiety response. Understanding pan-tissue mechanisms pathogenesis versus those which are tissue-specific, as well gauging their associated inter-individual variation owing to genetic background stochastic processes, paramount importance in syndromology. The BBSome a membrane-trafficking intraflagellar transport (IFT) adaptor...
Abstract Bardet-Biedl Syndrome (BBS) is a pleiotropic genetic disease caused by dysfunction of primary cilia. The immune system patients with BBS or another ciliopathy has not been investigated, most likely because hematopoietic cells do form However, there are multiple indications that the impairment processes typically associated cilia might influence compartment and immunity. In this study, we analyzed clinical data as well corresponding mouse model BBS4 deficiency. We uncovered have...
Abstract Bardet-Biedl Syndrome (BBS) is a pleiotropic ciliopathy caused by dysfunction of primary cilia. Most BBS patients carry mutations in one eight genes encoding for subunits protein complex, BBSome, which mediates the trafficking ciliary cargoes. Although, structure BBSome has been resolved recently, mechanism assembly this complicated complex living cells poorly understood. We generated large library human retinal epithelial cell lines deficient particular subunit and expressing...
Abstract Bardet-Biedl syndrome (BBS) is a pleiotropic ciliopathy caused by dysfunction of the BBSome, cargo adaptor essential for export transmembrane receptors from cilia. Although actin-dependent ectocytosis has been proposed as compensatory cellular process defective retrieval, underlying molecular mechanism poorly understood, particularly in context BBS pathology. In this study, we investigated how actin polymerization and are regulated within cilium. Our findings reveal that ciliary...
Abstract Bardet-Biedl syndrome (BBS) is an archetypal ciliopathy caused by dysfunction of primary cilia. BBS affects multiple tissues, including the kidney, eye and hypothalamic satiety response. Understanding pan-tissue mechanisms pathogenesis versus those which are tissue specific, gauging their associated inter-individual variation owing to genetic background stochastic processes, paramount importance in syndromology. The BBSome a membrane trafficking intraflagellar transport (IFT)...