Venera Kuci,1,2,* Lena Nordström,1,2,* Mats Jerkeman,3 Sara Ek1,2 1Department of Immunotechnology, Lund University, Lund, Sweden; 2CREATE Health, Lund University, Lund, Sweden; 3Department of Oncology, Lund University, Lund, Sweden *These authors contributed equally to this work Abstract: During recent years the neural transcription factor SOX11 has been established as an important biomarker for mantle cell lymphoma. SOX11 is both a diagnostic and prognostic antigen, and may potentially be used for treatment selection for younger patients, in relation to protocols including high dose chemotherapy. The molecular pathways involved are still not fully elucidated and, as SOX11 can interact with several co-transcription factors, functional assays need to be carefully designed to pinpoint SOX11-specific function in a defined cellular context. Furthermore, as SOX11 belongs to a large family of homologous proteins, analysis of SOX11 has been limited by the availability of specific antibodies for detection and pull-down. In this review, we discuss the emerging role of SOX11 in mantle cell lymphoma and discuss the potential impact in relation to tumorigenesis, diagnostics, prognostics, and therapy. Keywords: SOX11, mantle cell lymphoma, diagnostic