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SFB 1192

Projekt C1

Hamburg Glomerulonephritis Registry

Immune-mediated glomerular diseases are a common cause of end stage renal disease. However, despite huge experimental efforts over several decades, reliable biomarkers, preventive strategies and individualized therapies are still missing for most glomerular diseases. The difficulties in closing the translational circle between experimental kidney work and clinical application are often caused by a failed transfer of cell culture or animal data into the human context. Thus, we hypothesized that successful translational nephrology should be grounded on the availability of well characterized patient cohorts and biosamples including renal biopsies. Therefore, the Hamburg Glomerulonephritis Registry has been implemented as an integral part of the CRC initiative. Of note, already 530 patients with immune-mediated glomerular diseases have been included in the Hamburg Glomerulonephritis Registry allowing clinical, genetic and tissue information as well as biomaterials to be made available to all groups within the CRC. By this, the Registry facilitated several discoveries throughout the CRC. Particularly, the breakthrough findings leading to a better understanding of the pathophysiology of membranous nephropathy and therapy-relevant stratification of patients with this disease exemplify the enormous potential of the Hamburg Glomerulonephritis Registry. To further extend such patient relevant translational research, we aim to: 1. Continue the recruitment of patients with immunemediated glomerular diseases up to 1500 patients including prospective clinical data and biosamples. 2. Facilitate novel in depth clinical and tissue analysis (i.e. single cell techniques and machine learning, multiplex imaging, genome, epigenome and proteome analysis). 3. Identify, characterize and validate biomarkers for diagnosis, treatment response and prognosis of immune-mediated glomerular diseases. Collectively, the unique combination of clinical observation, informative patient cohorts, novel complex tissue analysis strategies, data integration and national and international collaborations should provide the ideal platform for CRC projects to catalyze novel biomarker discoveries and to cure immune-mediated glomerular diseases.

Publications with support of C1

Diagnostic role of renal biopsy in PLA2R1-antibody positive patients with nephrotic syndrome. 
Wiech T, Stahl RAK, Hoxha E (2019). 
Mod Pathol. In Print.

Genetics of membranous nephropathy.
Gupta S, Köttgen A, Hoxha E, Brenchley P, Bockenhauer D, Stanescu HC, Kleta R (2018).
Nephrol Dial Transplant 33:1493-1502

Antigen-Specific IgG Subclasses in Primary and Malignancy-Associated Membranous Nephropathy.
von Haxthausen F, Reinhard L, Pinnschmidt HO, Rink M, Soave A, Hoxha E*, Stahl RAK* (2018).
Front Immunol 9:3035. doi: 10.3389/fimmu.2018.03035. . *: equal contribution.

Bevacizumab-associated glomerular microangiopathy.
Person F, Rinschen MM, Brix SR, Wulf S, Noriega MLM, Fehrle W, Schmitz J, Schwarz A, Ivanyi P, Steinmetz OM, Reinhard L, Hoxha E, Zipfel PF, Bräsen JH, Wiech T (2018).
Mod Pathol doi: 10.1038/s41379-018-0186-4. [Epub ahead of print]

Organisation of lymphocytic infiltrates in ANCA-associated glomerulonephritis.
Brix SR, Noriega M, Herden EM, Goldmann B, Langbehn U, Busch M, Jabs WJ, Steinmetz OM, Panzer U, Huber TB, Stahl RAK, Wiech T (2018).
Histopathology 72:1093-1101.

IL-17C/IL-17 Receptor E Signaling in CD4+ T Cells Promotes TH17 Cell-Driven Glomerular Inflammation.
Krohn S, Nies JF, Kapffer S, Schmidt T, Riedel JH, Kaffke A, Peters A, Borchers A, Steinmetz OM, Krebs CF, Turner JE, Brix SR, Paust HJ, Stahl RAK, Panzer U (2018).
J Am Soc Nephrol 29:1210-1222.

Development and validation of a renal risk score in ANCA-associated glomerulonephritis.
Brix SR, Noriega M, Tennstedt P, Vettorazzi E, Busch M, Nitschke M, Jabs WJ, Özcan F, Wendt R, Hausberg M, Sellin L, Panzer U, Huber TB, Waldherr R, Hopfer H, Stahl RAK, Wiech T (2018).
Kidney Int 94:1177-1188.

The Most N-Terminal Region of THSD7A Is the Predominant Target for Autoimmunity in THSD7A-Associated Membranous Nephropathy.
Seifert L*, Hoxha E*, Eichhoff AM, Zahner G, Dehde S, Reinhard L, Koch-Nolte F, Stahl RAK, Tomas NM (2018).
J Am Soc Nephrol 29:1536-1548. *: equal contribution.

Immunohistochemical and serological characterization of membranous nephropathy in children and adolescents.
Dettmar AK, Wiech T, Kemper MJ, Soave A, Rink M, Oh J, Stahl RAK, Hoxha E (2018).
Pediatr Nephrol 33:463-472

A Heterologous Model of Thrombospondin Type 1 Domain-Containing 7A-Associated Membranous Nephropathy.
Tomas NM, Meyer-Schwesinger C, von Spiegel H, Kotb AM, Zahner G, Hoxha E, Helmchen U, Endlich N, Koch-Nolte F, Stahl RAK (2017).
J Am Soc Nephrol 28:3262-3277

THSD7A expression in human cancer.
Stahl PR, Hoxha E, Wiech T, Schröder C, Simon R, Stahl RA (2017).
Genes Chromosomes Cancer 56:314-327

An Indirect Immunofluorescence Method Facilitates Detection of Thrombospondin Type 1 Domain-Containing 7A-Specific Antibodies in Membranous Nephropathy.
Hoxha E, Beck LH Jr, Wiech T, Tomas NM, Probst C, Mindorf S, Meyer-Schwesinger C, Zahner G, Stahl PR, Schöpper R, Panzer U, Harendza S, Helmchen U, Salant DJ, Stahl RA (2017).
J Am Soc Nephrol 28:520-531

Autoimmune Renal Disease Is Exacerbated by S1P-Receptor-1-Dependent Intestinal Th17 Cell Migration to the Kidney.
Krebs CF, Paust HJ, Krohn S, Koyro T, Brix SR, Riedel JH, Bartsch P, Wiech T, Meyer-Schwesinger C, Huang J, Fischer N, Busch P, Mittrücker HW, Steinhoff U, Stockinger B, Perez LG, Wenzel UO, Janneck M, Steinmetz OM, Gagliani N, Stahl RAK, Huber S, Turner JE, Panzer U (2016).
Immunity 45:1078-1092.

Autoantibodies against thrombospondin type 1 domain-containing 7A induce membranous nephropathy.
Tomas NM*, Hoxha E*, Reinicke AT, Fester L, Helmchen U, Gerth J, Bachmann F, Budde K, Koch-Nolte F, Zahner G, Rune G, Lambeau G, Meyer-Schwesinger C§, Stahl RA§ (2016).
J Clin Invest 126:2519-2532. *§: equal contribution.

A Mechanism for Cancer-Associated Membranous Nephropathy.
Hoxha E, Wiech T, Stahl PR, Zahner G, Tomas NM, Meyer-Schwesinger C, Wenzel U, Janneck M, Steinmetz OM, Panzer U, Harendza S, Stahl RA (2016).
N Engl J Med 374:1995-1996