Check out this cool new paper from Mike Rogers lab @garvaninstitute.bsky.social out in @cp-immunity.bsky.social! Their new model of MKD deficiency (aka Hyper IgD syndrome) as a "prenylopathy": NK cell degranulation defect -> compensatory increase IFN gamma -> autoinflammation. Can Rx with JAKi!
NK cell dysfunction and interferon gamma production underlie autoinflammation in mevalonate kinase deficiency
The mechanisms linking defective protein prenylation to systemic inflammation in mevalonate kinase deficiency remain unclear. Munoz et al. reveal that mice and humans with MKD have defective NK cells,...
cell.com