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| Item Type: | Article |
|---|---|
| Title: | GIMAP6 regulates autophagy, immune competence, and inflammation in mice and humans |
| Creators: |
Yao, Y.  ORCID: https://orcid.org/0000-0002-8890-2409, Du Jiang, P. ORCID: https://orcid.org/0000-0002-9900-8277, Chao, B.N. ORCID: https://orcid.org/0000-0002-0355-2332, Cagdas, D. ORCID: https://orcid.org/0000-0003-2213-4627, Kubo, S. ORCID: https://orcid.org/0000-0001-9693-9263, Balasubramaniyam, A. ORCID: https://orcid.org/0000-0003-4168-8617, Zhang, Y. ORCID: https://orcid.org/0000-0002-6904-0372, Shadur, B. ORCID: https://orcid.org/0000-0003-0245-894X, NaserEddin, A. ORCID: https://orcid.org/0000-0001-6995-3262, Folio, L.R., Schwarz, B. ORCID: https://orcid.org/0000-0002-5894-953X, Bohrnsen, E. ORCID: https://orcid.org/0000-0003-4327-5242, Zheng, L. ORCID: https://orcid.org/0000-0002-8838-8665, Lynberg, M. ORCID: https://orcid.org/0000-0003-1371-3002, Gottlieb, S. ORCID: https://orcid.org/0000-0003-0550-4761, Leney-Greene, M.A. ORCID: https://orcid.org/0000-0002-3259-1728, Park, A.Y. ORCID: https://orcid.org/0000-0002-0130-1521, Tezcan, I. ORCID: https://orcid.org/0000-0001-7257-4554, Akdogan, A., Gocmen, R. ORCID: https://orcid.org/0000-0002-0223-9336, Onder, S. ORCID: https://orcid.org/0000-0002-5523-0669, Rosenberg, A., Soilleux, E.J. ORCID: https://orcid.org/0000-0002-4032-7249, Johnson, E. ORCID: https://orcid.org/0000-0001-8435-9472, Jackson, P.K. ORCID: https://orcid.org/0000-0002-1742-2539, Demeter, J., Chauvin, S.D. ORCID: https://orcid.org/0000-0003-0232-751X, Paul, F. ORCID: https://orcid.org/0000-0001-5181-3122, Selbach, M. ORCID: https://orcid.org/0000-0003-2454-8751, Bulut, H. ORCID: https://orcid.org/0000-0003-0262-6296, Clatworthy, M.R. ORCID: https://orcid.org/0000-0002-3340-9828, Tuong, Z.K. ORCID: https://orcid.org/0000-0002-6735-6808, Zhang, H., Stewart, B.J. ORCID: https://orcid.org/0000-0003-4522-0085, Bosio, C.M. ORCID: https://orcid.org/0000-0002-7332-9611, Stepensky, P. ORCID: https://orcid.org/0000-0002-4923-3660, Clare, S. ORCID: https://orcid.org/0000-0001-9368-6037, Ganesan, S. ORCID: https://orcid.org/0000-0003-1132-2016, Pascall, J.C. ORCID: https://orcid.org/0000-0002-9506-1441, Daumke, O. ORCID: https://orcid.org/0000-0002-6190-1414, Butcher, G.W. ORCID: https://orcid.org/0000-0002-3423-7124, McMichael, A.J. ORCID: https://orcid.org/0000-0002-9101-7478, Simon, A.K. ORCID: https://orcid.org/0000-0002-4077-7995 and Lenardo, M.J. ORCID: https://orcid.org/0000-0003-1584-468X
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| 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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