Cargando…
Oxysterol misbalance critically contributes to Wilson disease pathogenesis
Wilson disease (WD) is a metabolic disorder caused by inactivation of the copper-transporting ATPase 2 (ATP7B) and copper (Cu) overload in tissues. Excess Cu causes oxidative stress and pathologic changes with poorly understood mechanistic connections. In Atp7b(−/−) mice with established liver disea...
Autores principales: | Dev, Som, Muchenditsi, Abigael, Gottlieb, Aline, Deme, Pragney, Murphy, Sean, Gabrielson, Kathleen L., Dong, Yixuan, Hughes, Robert, Haughey, Norman J., Hamilton, James P., Lutsenko, Svetlana |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
American Association for the Advancement of Science
2022
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9581482/ https://www.ncbi.nlm.nih.gov/pubmed/36260680 http://dx.doi.org/10.1126/sciadv.adc9022 |
Ejemplares similares
-
Systemic deletion of Atp7b modifies the hepatocytes’ response to copper overload in the mouse models of Wilson disease
por: Muchenditsi, Abigael, et al.
Publicado: (2021) -
Wilson Disease: Update on Pathophysiology and Treatment
por: Dev, Som, et al.
Publicado: (2022) -
Copper
Capture in a Thioether-Functionalized Porous
Polymer Applied to the Detection of Wilson’s Disease
por: Lee, Sumin, et al.
Publicado: (2016) -
Urinary Copper Elevation in a Mouse Model of Wilson's Disease Is
a Regulated Process to Specifically Decrease the Hepatic Copper
Load
por: Gray, Lawrence W., et al.
Publicado: (2012) -
Structure of the Wilson disease copper transporter ATP7B
por: Bitter, Ryan M., et al.
Publicado: (2022)