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

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Item Type:Article
Title:GIMAP6 regulates autophagy, immune competence, and inflammation in mice and humans
Creators Name:Yao, Y. and Du Jiang, P. and Chao, B.N. and Cagdas, D. and Kubo, S. and Balasubramaniyam, A. and Zhang, Y. and Shadur, B. and NaserEddin, A. and Folio, L.R. and Schwarz, B. and Bohrnsen, E. and Zheng, L. and Lynberg, M. and Gottlieb, S. and Leney-Greene, M.A. and Park, A.Y. and Tezcan, I. and Akdogan, A. and Gocmen, R. and Onder, S. and Rosenberg, A. and Soilleux, E.J. and Johnson, E. and Jackson, P.K. and Demeter, J. and Chauvin, S.D. and Paul, F. and Selbach, M. and Bulut, H. and Clatworthy, M.R. and Tuong, Z.K. and Zhang, H. and Stewart, B.J. and Bosio, C.M. and Stepensky, P. and Clare, S. and Ganesan, S. and Pascall, J.C. and Daumke, O. and Butcher, G.W. and McMichael, A.J. and Simon, A.K. and Lenardo, M.J.
Abstract: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.
Keywords:Autophagy, Endothelial Cells, GTP Phosphohydrolases, Immunologic Deficiency Syndromes, Inflammation, Animals, Mice
Source:Journal of Experimental Medicine
ISSN:0022-1007
Publisher:Rockefeller University Press
Volume:219
Number:6
Page Range:e20201405
Date:6 June 2022
Official Publication:https://doi.org/10.1084/jem.20201405
PubMed:View item in PubMed

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