GIMAP6 regulates autophagy, immune competence, and inflammation in mice and humans

Yikun Yao, Pingdu Jiang, Brittany N. Chao, Deniz Cagdas, Satoshi Kubo, Arasu Balasubramaniyam, Yu Zhang, Bella Shadur, Adeeb Nasereddin, Les R. Folio, Benjamin Schwarz, Eric Bohrnsen, Lixin Zheng, Matthew Lynberg, Simone Gottlieb, Michael A. Leney-Greene, Ann Y. Park, Ilhan Tezcan, Ali Akdogan, Rahsan GocmenSevgen Onder, Avi Rosenberg, Elizabeth J. Soilleux, Errin Johnson, Peter K. Jackson, Janos Demeter, Samuel D. Chauvin, Florian Paul, Matthias Selbach, Haydar Bulut, Menna R. Clatworthy, Zewen K. Tuong, Hanlin Zhang, Benjamin J. Stewart, Catharine M. Bosio, Polina Stepensky, Simon Clare, Sundar Ganesan, Johnc Pascall, Oliver Daumke, Geoffreyw Butcher, Andrew J. McMichael, Anna Katharina Simon, Michael J. Lenardo

Research output: Contribution to journalArticlepeer-review


Inborn errors of immunity (IEIs) unveil regulatory pathways of human immunity. We describe a new IEI caused by mutations in the GTPase of the immune-associated protein 6 (GIMAP6) gene in patients with infections, lymphoproliferation, autoimmunity, and multiorgan vasculitis. Patients and Gimap6−/− mice show defects in autophagy, redox regulation, and polyunsaturated fatty acid (PUFA)–containing lipids. We find that GIMAP6 complexes with GABARAPL2 and GIMAP7 to regulate GTPase activity. Also, GIMAP6 is induced by IFN-γ and plays a critical role in antibacterial immunity. Finally, we observed that Gimap6−/− mice died prematurely from microangiopathic glomerulosclerosis most likely due to GIMAP6 deficiency in kidney endothelial cells.

Original languageEnglish (US)
Article numbere20201405
JournalJournal of Experimental Medicine
Issue number6
StatePublished - Jun 6 2022

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology


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