Ameliorating liver disease in an autosomal recessive polycystic kidney disease mouse model-Reference-Cited by-同舟云学术

Ameliorating liver disease in an autosomal recessive polycystic kidney disease mouse model

Published:2023-05-01 Issue:5 Volume:324 Page:G404-G414
ISSN:0193-1857
Container-title:American Journal of Physiology-Gastrointestinal and Liver Physiology
language:en
Short-container-title:American Journal of Physiology-Gastrointestinal and Liver Physiology

Author:

Yanda Murali K.¹^ORCID,Zeidan Adi¹,Cebotaru Liudmila¹^ORCID

Affiliation:

1. Departments of Medicine and Physiology, The Johns Hopkins University School of Medicine, Baltimore, Maryland, United States

Abstract

ARPKD is a multiorgan genetic disorder resulting in newborn morbidity and mortality. There is a critical need for new therapies to treat this disease. We show that persistent cholangiocytes proliferation occurs in a mouse model of ARPKD along with mislocalized CFTR and misregulated heat shock proteins. We found that VX-809, a CFTR modulator, inhibits proliferation and limits bile duct malformation. The data provide a therapeutic pathway for strategies to treat ADPKD.

Funder

HHS | NIH | National Institute of Diabetes and Digestive and Kidney Diseases

Publisher

American Physiological Society

Subject

Physiology (medical),Gastroenterology,Hepatology,Physiology

Link

https://journals.physiology.org/doi/pdf/10.1152/ajpgi.00255.2022

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