title: Systemic and neuronal inflammatory markers in a mouse model of mevalonate kinase deficiency: a strain-comparative study.
abstract: OBJECTIVE
  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.
  
  
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Institute for Maternal and Child Health - IRCCS Burlo Garofolo, Via dell'Istria, 65/1 - 34137 Trieste, Italy. giulio.kleiner@burlo.trieste.it.
authors.forename: Giulio
authors.initials: G
authors.lastname: Kleiner
authors.forename: Fulvio
authors.initials: F
authors.lastname: Celsi
authors.forename: Paola Maura
authors.initials: PM
authors.lastname: Tricarico
authors.forename: Serena
authors.initials: S
authors.lastname: Zacchigna
authors.forename: Sergio
authors.initials: S
authors.lastname: Crovella
authors.forename: Annalisa
authors.initials: A
authors.lastname: Marcuzzi
mesh_terms: D000119:Acetylmuramyl-Alanyl-Isoglutamine; D019386:Alendronate; D000818:Animals; D015415:Biomarkers / Q000097:blood; D002352:Carrier Proteins / Q000097:blood; D016207:Cytokines / Q000097:blood*; D004195:Disease Models, Animal; D006801:Humans; D058847:Inflammasomes / Q000097:blood; D018836:Inflammation Mediators / Q000097:blood; D008297:Male; D054078:Mevalonate Kinase Deficiency / Q000097:blood* / Q000139:chemically induced / Q000276:immunology; D051379:Mice; D008807:Mice, Inbred BALB C; D008810:Mice, Inbred C57BL; D000071199:NLR Family, Pyrin Domain-Containing 3 Protein; D013154:Spleen / Q000473:pathology
chemical_list: D015415:Biomarkers; D002352:Carrier Proteins; D016207:Cytokines; D058847:Inflammasomes; D018836:Inflammation Mediators; D000071199:NLR Family, Pyrin Domain-Containing 3 Protein; C492716:Nlrp3 protein, mouse; D000119:Acetylmuramyl-Alanyl-Isoglutamine; D019386:Alendronate
keywords: BALB/c; C57BL/6; Mevalonate kinase deficiency; alendronate; inflammasome; muramyl dipeptide