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Article

ISG15 deficiency features a complex cellular phenotype that responds to treatment with itaconate and derivatives.

Clinical and translational medicine - 1 Jul 2022

Waqas Syed Fakhar-Ul-Hassnain, Sohail Aaqib, Nguyen Ariane Hai Ha, Usman Abdulai, Ludwig Tobias, Wegner Andre, Malik Muhammad Nasir Hayat, Schuchardt Sven, Geffers Robert, Winterhoff Moritz, Merkert Sylvia, Martin Ulrich, Olmer Ruth, Lachmann Nico, Pessler Frank

Abstract excerpt

BACKGROUND: Congenital ISG15 deficiency is a rare autoinflammatory disorder that is driven by chronically elevated systemic interferon levels and predominantly affects central nervous system and skin. METHODS AND RESULTS: We have developed induced pluripotent stem cell-derived macrophages and endothelial cells as a model to study the cellular phenotype of ISG15 deficiency and identify novel treatments. ISG15-/-...

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Identifiers

PMID: 35842904
DOI: 10.1002/ctm2.931
PMCID: PMC9288839

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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