CFTR dysfunction predisposes to fibrotic liver disease in a murine model

Cystic fibrosis liver disease (CFLD) is a rapidly progressive biliary fibrosis, resembling primary sclerosing cholangitis that develops in 5–10% of patients with cystic fibrosis. Further research and evaluation of therapies are hampered by the lack of a mouse model for CFLD. Although primary scleros...

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Autores principales: Martin, Camilia R., Zaman, Munir M., Ketwaroo, Gyanprakash A., Bhutta, Abdul Q., Coronel, Emmanuel, Popov, Yury, Schuppan, Detlef, Freedman, Steven D.
Formato: Artigo
Lenguaje:Inglês
Publicado: American Physiological Society 2012
Materias:
Liver and Biliary Tract
Acceso en línea:https://ncbi.nlm.nih.gov/pmc/articles/PMC3423138/
https://ncbi.nlm.nih.gov/pubmed/22679000
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1152/ajpgi.00055.2012
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