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- Title
Hyper-IgD Syndrome: Caused by Deficiency on Ras Prenylation and Trained Immunity?
- Authors
Silva, Rafael Cardoso Maciel Costa
- Abstract
Hyper-IgD syndrome (HIDS) is an autoinflammatory disease characterized by physiological abnormalities such as skin rash, recurrent fever, and arthralgia. The disease is caused by mutations in the mevalonate kinase enzyme, which affects cholesterol and isoprenoid biosynthesis. The pathogenesis of HIDS is complex and involves multiple factors, including decreased MAPKs signaling, increased trained immunity, and activation of certain proteins. The increased levels of immunoglobulin D (IgD) in HIDS are likely due to the absence of MAPKs signaling from surface IgM. The author proposes using animal models and clinical studies to further evaluate the mechanisms and potential treatments for HIDS.
- Subjects
MEVALONATE kinase; ISOPRENYLATION; EXANTHEMA; SYNDROMES; AUTOINFLAMMATORY diseases; JOINT pain
- Publication
Journal of Clinical Immunology, 2023, Vol 43, Issue 8, p1740
- ISSN
0271-9142
- Publication type
Article
- DOI
10.1007/s10875-023-01548-x