Systemic and Neuronal Inflammatory Markers in a Mouse Model of Mevalonate Kinase Deficiency: a Strain-comparative Study

被引：0

作者：

Kleiner, Giulio ^{[1
]}

Celsi, Fulvio ^{[1
]}

Tricarico, Paola Maura ^{[2
]}

Zacchigna, Serena ^{[3
]}

Crovella, Sergio ^{[1
,2
]}

Marcuzzi, Annalisa ^{[1
]}

机构：

[1] Ist Ricovero & Cura Carattere Sci Burlo Garofolo, Inst Maternal & Child Hlth, Hlth Genet Unit, I-34137 Trieste, Italy

[2] Univ Trieste, Dept Med & Surg & Hlth Sci, Trieste, Italy

[3] Int Ctr Genet Engn & Biotechnol, Mol Med Lab, I-34012 Trieste, Italy

来源：

IN VIVO | 2013年 / 27卷 / 06期

关键词：

Mevalonate kinase deficiency; BALB/c; C57BL/6; alendronate; muramyl dipeptide; inflammasome; HYPERIMMUNOGLOBULINEMIA-D; NATURAL ISOPRENOIDS; NALP3; INFLAMMASOME; INHIBITION; ACTIVATION; CYTOKINES; ACIDURIA; MICE; LPS;

D O I：

暂无

中图分类号：

R-3 [医学研究方法]; R3 [基础医学];

学科分类号：

1001 ;

摘要：

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.

引用

页码：715 / 722

页数：8

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