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Systemic and neuronal inflammatory markers in a mouse model of mevalonate kinase deficiency: a strain-comparative
Giulio Kleiner1, Fulvio Celsi, Paola Maura Tricarico
1Institute for Maternal and Child Health - IRCCS Burlo Garofolo, Via dell'Istria, 65/1 - 34137 Trieste, Italy. giulio.kleiner@burlo.trieste.it.
Background/Aim:
There is a lack of reliable animal models for the study of the rare auto-inflammatory disease mevalonate kinase deficiency (MKD). The one most frequently used is a biochemical model, obtained by treating BALB/c mice in order to block the mevalonate pathway, thus attempting to reproduce the inflammatory pattern presented in patients. This study aims to assess the role played in pathology by the inflammasome and the reliability of this model.
Materials And Methods:
We mimicked MKD using two different mice strains (BALB/c and C57BL/6), evaluating typical inflammatory markers of MKD and inflammasome modulation.
Results:
Without significant differences, both strains exhibited a general MKD-like inflammation, including the modulation of the molecular platform inflammasome, mimicking the characteristics observed in human patients.
Conclusion:
Although with some limitations, the mouse model appears robust and suitable for studying MKD. Results do not seem to vary with the mouse strain used, and appear to be treatment-dependent. Finally, in vivo inflammasome activation was assessed for the first time here.
Insights
This study validates a mouse model for mevalonate kinase deficiency (MKD), a rare auto-inflammatory disease. The model effectively mimics human MKD inflammation and inflammasome activation, proving useful for research.
Area of Science:
- Immunology
- Genetics
- Pharmacology
Background:
- Mevalonate kinase deficiency (MKD) is a rare auto-inflammatory disease with limited reliable animal models.
- Current models often involve biochemical pathway blockade, necessitating validation.
Purpose of the Study:
- To assess the role of the inflammasome in MKD pathology.
- To evaluate the reliability of a biochemical mouse model for MKD.
Main Methods:
- MKD was mimicked in BALB/c and C57BL/6 mice.
- Inflammatory markers and inflammasome modulation were analyzed.
Main Results:
- Both mouse strains showed MKD-like inflammation and inflammasome modulation.
- These changes mirrored characteristics observed in human MKD patients.
Conclusions:
- The mouse model is robust and suitable for MKD research, independent of mouse strain.
- Treatment dependency was observed, and in vivo inflammasome activation was demonstrated for the first time.
