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Publication : GIMAP6 regulates autophagy, immune competence, and inflammation in mice and humans.

First Author  Yao Y Year  2022
Journal  J Exp Med Volume  219
Issue  6 PubMed ID  35551368
Mgi Jnum  J:326015 Mgi Id  MGI:7294325
Doi  10.1084/jem.20201405 Citation  Yao Y, et al. (2022) GIMAP6 regulates autophagy, immune competence, and inflammation in mice and humans. J Exp Med 219(6):e20201405
abstractText  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-gamma 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.
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