TY - GEN AU - Kaimori,Jun-Ya AU - Lin,Cheng-Chao AU - Outeda,Patricia AU - Garcia-Gonzalez,Miguel A AU - Menezes,Luis F AU - Hartung,Erum A AU - Li,Ao AU - Wu,Guanqing AU - Fujita,Hideaki AU - Sato,Yasunori AU - Nakanuma,Yasuni AU - Yamamoto,Satoko AU - Ichimaru,Naotsugu AU - Takahara,Shiro AU - Isaka,Yoshitaka AU - Watnick,Terry AU - Onuchic,Luiz F AU - Guay-Woodford,Lisa M AU - Germino,Gregory G TI - NEDD4-family E3 ligase dysfunction due to PKHD1/Pkhd1 defects suggests a mechanistic model for ARPKD pathobiology SN - 2045-2322 PY - 2019///0225 KW - Animals KW - Biomarkers KW - Cell Line KW - Disease Models, Animal KW - Enzyme Activation KW - Gene Expression KW - Humans KW - Intracellular Space KW - metabolism KW - Male KW - Mice KW - Mice, Knockout KW - Mice, Transgenic KW - Models, Biological KW - Mutation KW - Nedd4 Ubiquitin Protein Ligases KW - Polycystic Kidney, Autosomal Recessive KW - genetics KW - Protein Transport KW - Rats KW - Receptors, Cell Surface KW - deficiency KW - Signal Transduction KW - rho GTP-Binding Proteins N1 - Publication Type: Journal Article; Research Support, N.I.H., Extramural; Research Support, N.I.H., Intramural; Research Support, Non-U.S. Gov't UR - https://doi.org/10.1038/s41598-017-08284-4 ER -