25. April 2025

Immune Cells Drive Congenital Paralysis Disease Immune Cells Drive Congenital Paralysis Disease

A joint study conducted by the University of Bonn and the DZNE has confirmed early-stage brain inflammation in mice

Patients with spastic paraplegia type 15 develop movement disorders during adolescence that may ultimately require the use of a wheelchair. In the early stages of this rare hereditary disease the brain appears to play a major role by over-activating the immune system, as shown by a recent study published in the Journal of Experimental Medicine. The study was led by researchers at the University of Bonn and the German Center for Neurodegenerative Diseases (DZNE). These findings could also be relevant for Alzheimer's disease and other neurodegenerative conditions.

Microglia (magenta) - interacting with T cells (yellow) in the central nervous system of SPG15-deficient mice. Cell nuclei are shown in grey.
Microglia (magenta) - interacting with T cells (yellow) in the central nervous system of SPG15-deficient mice. Cell nuclei are shown in grey. © Dr. Stumm’s working group/University of Jena
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Spastic paraplegia type 15 is characterized by the progressive loss of neurons in the central nervous system that are responsible for controlling movement. Initial symptoms typically appear in late childhood, manifesting first in the legs in the form of uncontrollable twitching and paralysis. “What exactly causes these neurons to die is still not fully understood,” explains Professor Elvira Mass from the LIMES Institute at the University of Bonn. “In this study, we investigated the potential role of the immune system in this process.”

Professor Mass and Dr. Marc Beyer from the DZNE, together with Professor Ralf Stumm from University Hospital Jena, served as the study’s lead investigators, bringing together extensive experience to study this rare hereditary disease. The condition is triggered by a defect in the so-called SPG15 gene, which contains instructions for building a protein. But due to that defect, the protein cannot be produced.

Severe inflammation preceding the onset of cell damage

In their experiments the researchers used mice that shared the same genetic defect. “There was existing evidence that inflammatory processes in the brain play a role in development of the disease,” Dr. Beyer explains, “So we studied microglia, which are ...

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The authors would like to thank the Flow Cytometry Core Facility of the Mathematical and Natural Science Faculty at the University of Bonn for providing support and instrumentation

Alexander Frolov et al.: Microglia and CD8+ T cell activation precede neuronal loss in a murine model of spastic paraplegia 15; Journal of Experimental Medicine; DOI: https://doi.org/10.1084/jem.20232357

Prof. Dr. Elvira Mass
Life & Medical Sciences Institute (LIMES)
University of Bonn
Phone: +49 228 73-62848
Email: elvira@uni-bonn.de

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