Johannes Raedler
A5074059593- Immune Response and Inflammation
- interferon and immune responses
- Immunodeficiency and Autoimmune Disorders
- Immune Cell Function and Interaction
- Neutrophil, Myeloperoxidase and Oxidative Mechanisms
- Virology and Viral Diseases
- Cystic Fibrosis Research Advances
- Hematopoietic Stem Cell Transplantation
- T-cell and B-cell Immunology
- Acute Lymphoblastic Leukemia research
- Cytomegalovirus and herpesvirus research
- Viral Infections and Vectors
- Parvovirus B19 Infection Studies
- CAR-T cell therapy research
- Virus-based gene therapy research
- Chronic Obstructive Pulmonary Disease (COPD) Research
- Viral Infectious Diseases and Gene Expression in Insects
- Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
- Blood disorders and treatments
- Respiratory viral infections research
- Acute Myeloid Leukemia Research
- Viral Infections and Outbreaks Research
Ludwig-Maximilians-Universität München
2020-2024
LMU Klinikum
2021
Patients with autoimmune polyendocrinopathy syndrome type 1 (APS-1) caused by autosomal recessive AIRE deficiency produce autoantibodies that neutralize I interferons (IFNs)1,2, conferring a predisposition to life-threatening COVID-19 pneumonia3. Here we report patients NIK or RELB deficiency, specific of autosomal-dominant NF-κB2 also have neutralizing against IFNs and are at higher risk getting pneumonia. In these found only in individuals who heterozygous for variants associated both...
Hyperactive TLR7 signaling has long been appreciated as driver of autoimmune disease in mouse models. Recently, gain-of-function mutations were identified a monogenic cause human lupus. is an intracellular transmembrane receptor, sensing RNA breakdown products within late endosomes. Here, we show that endosome dysfunction leads to unrestricted and associated with The endosomal BORC complex together the small GTPase Arl8b controls levels by regulating receptor turnover. This requires direct...
Rubella virus (RuV) has recently been found in association with granulomatous inflammation of the skin and several internal organs patients inborn errors immunity (IEI). The cellular tropism molecular mechanisms RuV persistence pathogenesis select immunocompromised hosts are not clear. We provide clinical, immunological, virological, histological data on a cohort 28 broad spectrum IEI RuV-associated granulomas nine extracutaneous tissues to further delineate this relationship. Combined...
Patients with inherited CARMIL2 or CD28 deficiency have defective T cell signaling, but their immunological and clinical phenotypes remain largely unknown. We show that only one of three isoforms is produced functional across leukocyte subsets. Tested mutant alleles from 89 patients 52 families impair canonical NF-κB not AP-1 NFAT activation in cells stimulated via CD28. Like CD28-deficient patients, CARMIL2-deficient display recalcitrant warts low blood counts CD4+ CD8+ memory TREGs. Unlike...
Replication competent vesicular stomatitis virus (VSV) is the basis of a vaccine against Ebola and VSV strains are developed as oncolytic viruses. Both functions depend on ability to induce adequate amounts interferon-α/β. It therefore important understand how triggers interferon responses. activates innate immunity via retinoic acid-inducible gene I (RIG-I), sensor for viral RNA. Our results show that needs replicate robust response. Analysis RIG-I-associated RNA identified copy-back...
Background Relapsed/refractory B-precursor acute lymphoblastic leukemia (BCP-ALL) remains a major therapeutic challenge in pediatric hematology. Chimeric antigen receptor (CAR) T cells targeting CD19 have shown remarkable initial response rates BCP-ALL patients, while long-term control rate is only about 50%. So far, main mechanisms of relapse after CD19-CAR T-cell therapy been either insufficient CAR persistence vivo or loss surface CD19. Case Report Here, we report an exceptional...
Acute bronchiolitis is a common disease of infants affecting the small airways. Rarely, acute may occur in adolescents and adults. Here, we present four unrelated adolescent patients with severe clinical presentation unique CT imaging extensive tree-in-bud pattern, representing rare phenotype diffuse panbronchiolitis. This characteristic pattern caused by inhalation injury from waterpipes, smoked tobacco, cannabinoids must be differentiated e-cigarette or vaping product-use-associated lung...
Abstract Bi-allelic variants in the dedicator of cytokinesis 8 ( DOCK8 ) gene cause a combined immunodeficiency, characterized by recurrent sinopulmonary and skin infections, food allergies, eczema, eosinophilia, elevated IgE. Long-term outcome is poor given susceptibility to malignancy, vascular complications. Allogeneic hematopoietic stem cell transplantation currently only curative treatment option has shown promising outcome. The impact mixed chimerism on long-term unclear. We reasoned...
Abstract Hyperactive Toll-like receptor (TLR) 7 signaling has long been appreciated as a driver of autoimmune disease by breaking tolerance to self-nucleic acids in mouse models 1–5 . Recently, mutations TLR7 or its associated regulator UNC93B1 6, , were identified monogenic causes human lupus; the unifying feature these being gain-of-function. is an intracellular transmembrane receptor, sensing RNA breakdown products within late endosomes 8, 9 Hence, function depends on intricate transport...