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| Item Type: | Article |
|---|---|
| Title: | GIMAP6 regulates autophagy, immune competence, and inflammation in mice and humans |
| Creators Name: | Yao, Y., Du Jiang, P., Chao, B.N., Cagdas, D., Kubo, S., Balasubramaniyam, A., Zhang, Y., Shadur, B., NaserEddin, A., Folio, L.R., Schwarz, B., Bohrnsen, E., Zheng, L., Lynberg, M., Gottlieb, S., Leney-Greene, M.A., Park, A.Y., Tezcan, I., Akdogan, A., Gocmen, R., Onder, S., Rosenberg, A., Soilleux, E.J., Johnson, E., Jackson, P.K., Demeter, J., Chauvin, S.D., Paul, F., Selbach, M., Bulut, H., Clatworthy, M.R., Tuong, Z.K., Zhang, H., Stewart, B.J., Bosio, C.M., Stepensky, P., Clare, S., Ganesan, S., Pascall, J.C., Daumke, O., Butcher, G.W., McMichael, A.J., 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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