NEDD4-family E3 ligase dysfunction due to PKHD1/Pkhd1 defects suggests a mechanistic model for ARPKD pathobiology. [electronic resource]
- Scientific reports 08 2017
- 7733 p. digital
Publication Type: Journal Article; Research Support, N.I.H., Extramural; Research Support, N.I.H., Intramural; Research Support, Non-U.S. Gov't
2045-2322
10.1038/s41598-017-08284-4 doi
Animals Biomarkers Cell Line Disease Models, Animal Enzyme Activation Gene Expression Humans Intracellular Space--metabolism Male Mice Mice, Knockout Mice, Transgenic Models, Biological Mutation Nedd4 Ubiquitin Protein Ligases--metabolism Polycystic Kidney, Autosomal Recessive--genetics Protein Transport Rats Receptors, Cell Surface--deficiency Signal Transduction rho GTP-Binding Proteins--metabolism