A paper published in the journal Cell Reports describes the development of a new system based on stem cells to generate neurofibromas, tumors of the peripheral nervous system. These tumors develop in people who have a very prevalent hereditary disease, 1 in 3000 people, called Neurofibromatosis type 1, and are the main cause of death among these patients.
Until now, there was not a good model of human neurofibromas in mice to test new treatments. This work, led by the Hereditary Cancer group of the IGTP, and with the participation of researchers from the regenerative medicine program of IDIBELL and the P-CMRC, researchers from the Oncobell cancer research program of IDIBELL and ICO represents an important leap in the modeling of these tumors.
The originality of the work lies in the use of a 3D culture system, made up of Schwann cells, derived from human stem cells, and fibroblasts, isolated from patient tumors, the two main cell types of neurofibromas. This model can generate many spheres at the same time, and therefore, it is possible to evaluate multiple drugs. Furthermore, when these spheres are implanted in the sciatic nerve of a mouse, they can generate human neurofibromas, showing that they represent a reliable model.
Finally, single-cell analysis was used to study the cellular heterogeneity of patient neurofibromas. This has permitted them to have a clearer and more complete image of the cellular composition, helping to understand these tumors and design new potential drugs.
This work has also had the participation of the Center for Genomic Regulation (CRG-CNAG) and the Pathological Anatomy Service of the Vall d’Hebron Hospital.
The Bellvitge Biomedical Research Institute (IDIBELL) is a biomedical research center created in 2004. It is participated by the Bellvitge University Hospital and the Viladecans Hospital of the Catalan Institute of Health, the Catalan Institute of Oncology, the University of Barcelona and the City Council of L’Hospitalet de Llobregat.
IDIBELL is a member of the Campus of International Excellence of the University of Barcelona HUBc and is part of the CERCA institution of the Generalitat de Catalunya. In 2009 it became one of the first five Spanish research centers accredited as a health research institute by the Carlos III Health Institute. In addition, it is part of the “HR Excellence in Research” program of the European Union and is a member of EATRIS and REGIC. Since 2018, IDIBELL has been an Accredited Center of the AECC Scientific Foundation (FCAECC).