Article
Autoinhibition and regulation by phosphoinositides of ATP8B1, a human lipid flippase associated with intrahepatic cholestatic disorders.
eLife - 13 Apr 2022
Dieudonné Thibaud, Herrera Sara Abad, Laursen Michelle Juknaviciute, Lejeune Maylis, Stock Charlott, Slimani Kahina, Jaxel Christine, Lyons Joseph A, Montigny Cédric, Pomorski Thomas Günther, Nissen Poul, Lenoir Guillaume
Abstract excerpt
P4-ATPases flip lipids from the exoplasmic to the cytosolic leaflet, thus maintaining lipid asymmetry in eukaryotic cell membranes. Mutations in several human P4-ATPase genes are associated with severe diseases, for example in ATP8B1 causing progressive familial intrahepatic cholestasis, a rare inherited disorder progressing toward liver failure. ATP8B1 forms a binary complex with CDC50A and displays a broad...
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