A5067373544- Complement system in diseases
- Blood groups and transfusion
- Coagulation, Bradykinin, Polyphosphates, and Angioedema
- Renal Diseases and Glomerulopathies
- Monoclonal and Polyclonal Antibodies Research
- Platelet Disorders and Treatments
- Vector-borne infectious diseases
- Erythrocyte Function and Pathophysiology
- Liver Disease and Transplantation
- Glycosylation and Glycoproteins Research
- Immune Cell Function and Interaction
- Renal Transplantation Outcomes and Treatments
- Xenotransplantation and immune response
- Immunotherapy and Immune Responses
- Viral Infections and Vectors
- Organ Transplantation Techniques and Outcomes
- Mechanical Circulatory Support Devices
- Toxin Mechanisms and Immunotoxins
- Immunodeficiency and Autoimmune Disorders
- Hemoglobinopathies and Related Disorders
- Blood Coagulation and Thrombosis Mechanisms
- Diabetes and associated disorders
- Sepsis Diagnosis and Treatment
- Bacterial Infections and Vaccines
- Hemophilia Treatment and Research
Heidelberg University
2014-2024
University of Zurich
2022
Cold Spring Harbor Laboratory
2022
University Hospital Heidelberg
1999-2020
Essen University Hospital
2016
University of Duisburg-Essen
2016
Universität Hamburg
2016
Institute of Immunology
1990-2015
Heidelberg University
2014-2015
Aalborg University Hospital
2010
To understand immune evasion mechanisms of Borrelia burgdorferi we compared serum-resistant B. afzelii and serum-sensitive garinii isolates for their capacity toacquire human complement regulators. Here demonstrate that the two borrelial genospecies show different binding important regulators, FHL-1/reconectin Factor H. All bound also H, all analyzed showed no or a significantly lower activity. Using recombinant deletion mutants, domains were localized to C terminus short consensus repeats...
The etiologic agent of Lyme disease, Borrelia burgdorferi, is capable circumventing the immune defense a variety potential vertebrate hosts. Previous work has shown that interaction host-derived complement regulators, factor H and H-like protein 1 (FHL-1), with up to five regulator-acquiring surface proteins (CRASPs) expressed by resistant B. burgdorferi sensu lato isolates conferred resistance. In addition expression CRASP-1 directly correlated resistance species. This describes functional...
Atypical hemolytic uremic syndrome (aHUS) is associated with a congenital or acquired dysregulation of the complement alternative pathway that leads to continuous activation on host cells causing inflammation and damage. Eculizumab, humanized mAb against protein C5, inhibits terminal pathway.We report an adolescent relapsing unclassified aHUS. On admission, high plasma creatinine level indicated poor prognosis, hemodialysis had be started. Plasma exchanges were initially effective...
The renal disorder C3 glomerulopathy with dense deposit disease (C3G-DDD) pattern results from complement dysfunction and primarily affects children young adults. There is no effective treatment, patients often progress to end-stage failure. A small fraction of C3G-DDD cases linked factor H or gene mutations as well autoantibodies have been reported. Here, we examined an index family 2 identified a chromosomal deletion in the H–related (CFHR) cluster. This resulted expression hybrid...
Catastrophic antiphospholipid syndrome (CAPS) is a rare but devastating complication in patients with (APS) high morbidity and mortality.We describe case of 30-year old female patient immunoglobulin A (IgA) deficiency who underwent splenectomy because idiopathic thrombocytopenic thrombocytopenia. Subsequently, an APS finally systemic lupus erythematosus was diagnosed. After uncomplicated pregnancy that terminated by cesarean section, the developed severe CAPS cerebral, myocardial, renal,...
Various complement-mediated renal disorders are treated currently with the complement inhibitor eculizumab. By blocking cleavage of C5, this monoclonal antibody prevents cell damage caused by inflammation. We included 23 patients atypical haemolytic uraemic syndrome (aHUS, n = 12), C3 glomerulopathies (C3G, 9) and acute antibody-mediated graft rejection (AMR, 2), eculizumab in 12 hospitals Germany. explored course activation biomarkers benefit therapeutic drug monitoring Complement was...
Summary Borrelia burgdorferi , the aetiological agent of Lyme disease, employs sophisticated means to survive in diverse mammalian hosts. Recent studies demonstrated that acquisition complement regulators factor H and H‐like protein‐1 (FHL‐1) allows spirochetes resist complement‐mediated killing. Serum‐resistant B. express up five distinct regulator‐acquiring surface proteins (CRASPs) bind and/or FHL‐1. In this study we have identified characterized one those proteins, named BbCRASP‐2....
Abstract The causative agents of Lyme disease, Borrelia burgdorferi s.s., B. garinii , and afzelii differ in their susceptibility to complement‐mediated lysis. This phenomenon apparently depends on the expression proteins termed complement regulator‐acquiring surface (CRASP) binding inhibitory plasma factor H FHL‐1. To characterize these bacterial more detail we have now isolated from a library novel H‐binding protein. In accordance with our previous studies this protein was BbCRASP‐3...
SUMMARY Nucleated cells employ several strategies to evade killing by homologous complement. We studied complement resistance in the human carcinoma cell lines (CA) T47D (mammary), SKOV3 (ovarian), and PC-3 (prostate) with emphasis on following mechanisms of defense: 1. Expression shedding membrane regulatory proteins (mCRP) CD46, CD55 CD59; 2. Resistance based protein phosphorylation; 3. Cell surface expression sialic acid residues; 4. Desensitization upon exposure sublytic doses. Anti-mCRP...
SUMMARY In vivo and in vitro studies have shown that complement activation plays an important role the pathogenesis of adult respiratory distress syndrome (ARDS). a prospective study polytrauma patients at risk ARDS (n= 38) parameters were determined over period 14 days serial plasma samples (obtained every 6 h during first 48 h). Polytrauma induced rapid remarkable activation. Low levels proteins C3, C4, C1 inhibitor (Cl INH) factor I H indicated consumption all patients. Elevated C3a few...