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Here you will find recent publication of Diana Pauly and her group members about complement system in vision research.

Josef Biber, Yassin Jabri, Sarah Glänzer, Aaron Dort, Patricia Hoffelner, Christoph Q. Schmidt, Oliver Bludau, Diana Pauly*, Antje Grosche*

2024, Journal of Neuroinflammation

*These authors share senior authorship.

This study investigates the therapeutic potential of adeno-associated viruses (AAVs) engineered to express truncated human complement factor H (FH) variants in treating retinal diseases exacerbated by complement system overactivity. Following ischemic injury in mouse retinas, the AAVs targeted gliotic Müller cells, resulting in decreased complement activation, improved microglia activity, and morphological recovery, indicating the promise of this gene therapy approach for retinal conditions lacking effective treatments.

Hannah N Wolf, Veronika Ehinger, Larissa Guempelein, Tania Kuempfel, Joachim Havla, Diana Pauly

2023, Current Issues in Molecular Biology

We have added the most important results and take aways in a short blog entry which you can find here.

This exploratory study analyzed the effects of NMOSD immunoglobulins (IgG) on mouse retinal explants, revealing that NMOSD IgG increased gliosis and decreased chemokine release, potentially altering the retinal cellular environment and contributing to retinal degeneration independent of relapse activity. These findings suggest that NMOSD may cause primary retinopathy through mechanisms involving retinal glial cells.

Randy Zauhar, Josef Biber, Yassin Jabri, Mijin Kim, Jian Hu, Lew Kaplan, Anna M. Pfaller, Nicole Schäfer, Volker Enzmann, Ursula Schlötzer-Schrehardt, Tobias Straub, Stefanie M. Hauck, Paul D. Gamlin, Michael B. McFerrin, Jeffrey Messinger, Christianne E. Strang, Christine A. Curcio, Nicholas Dana, Antje Grosche*, Mingyao Li*, Dwight Stambolian* and Diana Pauly*

2022, Frontiers in Immunology

*These authors share senior authorship.

This study highlights the regional differences in cellular expression of complement proteins within the retina and its supporting tissues, noting significant variations between the macular and peripheral areas. These findings suggest that the location within the retina is crucial for understanding the role of complement in retinal diseases.

Schäfer N, Rasras A, Ormenisan D, Amslinger S, Enzmann V, Jägle H, Pauly D.

2021, Frontiers in Immunology

This study reveals that Complement Factor H-Related 3 (FHR-3) binds to oxidative stress epitopes, competes with Factor H, and is internalized by retinal pigment epithelial (RPE) cells, leading to increased expression of complement components and receptors. FHR-3 induces a pro-inflammatory microenvironment via inflammasome activation and cytokine secretion in RPE cells, suggesting its role as a trigger for complement activation in degenerative diseases and a potential therapeutic target.

Poppelaars F, Goicoechea de Jorge E, Jongerius I, Baeumner AJ, Steiner MS, Józsi M, Toonen EJM, Pauly D; SciFiMed consortium.

2021, Frontiers in Immunology

This perspective review highlights the critical role of the human Factor H (FH) protein family in various diseases characterized by complement dysregulation, emphasizing the need to address major challenges such as functional role controversies, quantification discrepancies, and the lack of validated tools and suitable animal models. The authors advocate for a strong multidisciplinary approach, facilitated by collaborations like the SciFiMed Consortium, to comprehensively analyze and understand the FH protein family.

Enzbrenner A, Zulliger R, Biber J, Pousa AMQ, Schäfer N, Stucki C, Giroud N, Berrera M, Kortvely E, Schmucki R, Badi L, Grosche A, Pauly D*, Enzmann V*.

2021, International Journal of Molecular Science

*These authors share senior authorship.

Jabri Y, Biber J, Diaz-Lezama N, Grosche A, Pauly D.

2020, International Journal of Molecular Science

Schäfer N, Wolf HN, Enzbrenner A, Schikora J, Reichenthaler M, Enzmann V, Pauly D.

2020, Antioxidants

Mietzner R, Kade C, Froemel F, Pauly D, Stamer WD, Ohlmann A, Wegener J, Fuchshofer R, Breunig M.

2020, Pharmaceutics

Pauly D*, Agarwal D*, Dana N, Schäfer N, Biber J, Wunderlich KA, Jabri Y, Straub T, Zhang NR, Gautam AK, Weber BHF, Hauck SM, Kim M, Curcio CA, Stambolian D, Li M, Grosche A.

2019, Cell Reports

* These authors share first authorship.

Trakkides TO, Schäfer N, Reichenthaler M, Kühn K, Brandwijk RJMGE, Toonen EJM, Urban F, Wegener J, Enzmann V, Pauly D.

2019, Antioxidants

von Berg L, Stern D, Pauly D, Mahrhold S, Weisemann J, Jentsch L, Hansbauer EM, Müller C, Avondet MA, Rummel A, Dorner MB, Dorner BG.

2019, Scientific Reports

Schäfer N, Grosche A, Schmitt SI, Braunger BM, Pauly D.

2017, Frontiers of Molecular Neuroscience

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