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.
journal_name
Cell Repjournal_title
Cell reportsauthors
Yang MY,Hilton MB,Seaman S,Haines DC,Nagashima K,Burks CM,Tessarollo L,Ivanova PT,Brown HA,Umstead TM,Floros J,Chroneos ZC,St Croix Bdoi
10.1016/j.celrep.2013.04.019subject
Has Abstractpub_date
2013-05-30 00:00:00pages
1457-64issue
5issn
2211-1247pii
S2211-1247(13)00201-5journal_volume
3pub_type
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