A14 | Immunological dissection of cell death-induced inflammation in human disease
Prof. Dr. Hirotsugu Oda
Institute for Systemic Inflammation Research
ISEF, University of Lübeck
Contact: hirotsugu.oda(at)uni-luebeck(dot)de
For more information please visit: Oda Lab
Abstract
This project is funded within the 2nd funding period of the SFB 1403, starting from 2024
Systemic Autoinflammatory Diseases (SAIDs) are a group of human disorders characterized by genetic hyperactivation of innate immunity, resulting in intractable inflammatory manifestations such as prolonged fever, rash, arthritis and colitis. Currently the clinical outcome of SAIDs is still discouraging, and there is an urgent need to discover new molecular targeted therapies. We will use a transdisciplinary approach by combining our experiences in clinical medicine, human genetics, bioinformatics and molecular immunology, to elucidate the contribution of cell death dysregulations as an important underlying pathomechanism of human SAIDs.
Recent Publications
2026
Awan FN, Zulfiqar S, Eiman L, Asif M, Hussain MS, Dahl N, Baig SM, Oda H. Clinical and Molecular Characterization of Pakistani Mucopolysaccharidosis Families with SGSH and GALNS Deficiencies. Genes (Basel). 2026 Mar 31;17(4):401. doi: 10.3390/genes17040401. PMID: 42074519; PMCID: PMC13116815.
Project A14 Publications 2nd Funding Period 2024 - 2027
2025
Oda H, Annibaldi A, Kastner DL, Aksentijevich I. Genetic Regulation of Cell Death: Insights from Autoinflammatory Diseases. Annu Rev Immunol. 2025 Apr;43(1):313-342. doi: 10.1146/annurev-immunol-090222-105848. PMID: 40279314.
2024
Oda H, Manthiram K, Chavan PP, Rieser E, Veli Ö, Kaya Ö, Rauch C, Nakabo S, Kuehn HS, Swart M, Wang Y, Çelik NI, Molitor A, Ziaee V, Movahedi N, Shahrooei M, Biallelic human SHARPIN loss of function induces autoinflammation and immunodeficiency. Nat Immunol. 2024 Apr 12. doi: 10.1038/s41590-024-01817-w. Epub ahead of print. PMID: 38609546.
Project related Publications
2017 - 2022
Oda H, Manthiram K, Pimpale Chavan P, Nakabo S, Kuehn HS, …, Lalaoi, N., Aksentijevich, I., and Kastner, D.L. (2022). Human LUBAC deficiency leads to autoinflammation and immunodeficiency by dysregulation in TNF-mediated cell death. medRxiv 2022.11.09.22281431; doi: https://doi.org/10.1101/2022.11.09.22281431
Oda, H., and Kastner, D.L. (2017). Genomics, Biology, and Human Illness: Advances in the Monogenic Autoinflammatory Diseases. Rheum Dis Clin North Am 43, 327-345.
Lalaoui, N*., Boyden, S.E*., Oda, H*., Wood, G.M., Stone, D.L., Chau, D., Liu, L., Stoffels, M., Kratina, T., Lawlor, K.E., …, Gadina, M., Kastner, D.L., and Silke, J. (2020). Mutations that prevent caspase cleavage of RIPK1 cause autoinflammatory disease. Nature 577, 103-108. * equal contribution
Oda, H., Beck, D.B., Kuehn, H.S., Sampaio Moura, N., Hoffmann, P., Ibarra, M., Stoddard, J., Tsai, W.L., Gutierrez-Cruz, G., Gadina, M., …, Kastner, D.L., Notarangelo, L.D., and Aksentijevich, I. (2019). Second Case of HOIP Deficiency Expands Clinical Features and Defines Inflammatory Transcriptome Regulated by LUBAC. Front Immunol 10, 479.