Ishikawa S et al. – These findings suggest that Systemic juvenile idiopathic arthritis (sJIA) is not only an immunological disease but also a metabolic disease involving mitochondria disorder. Methods
Aim was to clarify the cellular functional abnormality in sJIA
Gene expression profile was analyzed in peripheral leukocytes from 51 pts with sJIA, 6 pts with poly-articular type JIA (polyJIA) and 8 healthy children utilizing DNA microarrays
Gene ontology analysis and network analysis were performed on the genes differentially expressed in sJIA
Results
3,491 genes were differentially expressed in pts with sJIA vs healthy individuals
They were functionally categorized mainly into a defense-response group and a metabolism group suggesting the possible abnormalities in these functions
In the defense-response group, molecules predominantly constituting IFN-γ and TNF network cascades were up-regulated
In the metabolism group, oxidative phosphorylation-related genes were down-regulated, suggesting a mitochondrial disorder
Expression of mitochondrial DNA-encoded genes including cytochrome c oxidase (MT-CO) 1 and MT-CO2 were suppressed in pts with sJIA vs pts with polyJIA or healthy children
However, nuclear DNA-encoded cytochrome c oxidases were intact
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