@article{cb0ef9e5c4e342769a7ecc15f7e38fdb,
title = "Essential Regulation of Lung Surfactant Homeostasis by the Orphan G Protein-Coupled Receptor GPR116",
abstract = "GPR116 is an orphan seven-pass transmembrane receptor whose function has been unclear. Global disruption of the Gpr116 gene in mice revealed an unexpected, critical role for this receptor in lung surfactant homeostasis, resulting in progressive accumulation of surfactant lipids and proteins in the alveolar space, labored breathing, and a reduced lifespan. GPR116 expression analysis, bone marrow transplantation studies, and characterization of conditional knockout mice revealed that GPR116 expression in ATII cells is required for maintaining normal surfactant levels. Aberrant packaging of surfactant proteins with lipids in the Gpr116 mutant mice resulted in compromised surfactant structure, function, uptake, and processing. Thus, GPR116 plays an indispensable role in lung surfactant homeostasis with important ramifications for the understanding and treatment of lung surfactant disorders.",
author = "Yang, {Mi Young} and Hilton, {Mary Beth} and Steven Seaman and Haines, {Diana C.} and Kunio Nagashima and Burks, {Christina M.} and Lino Tessarollo and Ivanova, {Pavlina T.} and Brown, {H. Alex} and Umstead, {Todd M.} and Joanna Floros and Chroneos, {Zissis C.} and Brad St.Croix",
note = "Funding Information: This manuscript is dedicated to the memory of our dear friend and colleague Dr. Jo Rae Wright, who provided helpful advice and SFTPC-GFP mice for these studies. We thank Brigid L.M. Hogan and Jason R. Rock for providing SFTPC-Cre mice. This research was supported by the Center for Cancer Research Intramural Program, National Cancer Institute (NCI), National Institutes of Health, a part of the U.S. Department of Health and Human Services, and with federal funds from the NCI under contract nos. HHSN261200800001E and NIH HL34788. The content of this publication does not necessarily reflect the views or policies of the DHHS, nor does mention of trade names, commercial products, or organizations imply endorsement by the U.S. Government. Partial support for the lipidomic analysis was provided by an award from the NIGMS (U54 GM069338 to H.A.B.). ",
year = "2013",
month = may,
day = "30",
doi = "10.1016/j.celrep.2013.04.019",
language = "English (US)",
volume = "3",
pages = "1457--1464",
journal = "Cell Reports",
issn = "2211-1247",
publisher = "Cell Press",
number = "5",
}