Hedgehog signaling: implications in liver pathophysiology

RK Dutta, JH Jun, K Du, AM Diehl - Seminars in Liver Disease, 2023 - thieme-connect.com
The purpose of this review is to summarize current knowledge about the role of the
Hedgehog signaling pathway in liver homeostasis and disease. Hedgehog is a …

Van Gogh-like 2 is essential for the architectural patterning of the mammalian biliary tree

M Raab, E Christodoulou, R Krishnankutty… - Journal of …, 2024 - Elsevier
Background & Aims In the developing liver, bipotent epithelial progenitor cells undergo
lineage segregation to form hepatocytes, which constitute the bulk of the liver parenchyma …

What's new in pediatric genetic cholestatic liver disease: advances in etiology, diagnostics and therapeutic approaches

M Pinon, BM Kamath - Current Opinion in Pediatrics, 2024 - journals.lww.com
Next-generation sequencing (NGS) technologies have revolutionized diagnostic
approaches, while discovery of new intracellular signaling pathways show promise in …

Amyloid precursor protein as a fibrosis marker in infants with biliary atresia

JC Kamp, O Madadi-Sanjani, M Uecker, C Werlein… - Pediatric …, 2024 - nature.com
Background Biliary atresia (BA) is a rare condition of unknown origin in newborns with
jaundice. In BA bile ducts are non-functional, causing neonatal cholestasis and following …

Impact of gallbladder hypoplasia on hilar hepatic ducts in biliary atresia

N Miyazaki, S Takami, M Uemura, H Oiki… - Communications …, 2024 - nature.com
Background Biliary atresia (BA) is an intractable disease of unknown cause that develops in
the neonatal period. It causes jaundice and liver damage due to the destruction of …

Kasai Portoenterostomy, Successful Liver Transplantation, and Immunosuppressive Therapy for Biliary Atresia in a Female Baby: A Case Report

L Shan, F Wang, D Zhai, X Meng, J Liu… - Journal of Inflammation …, 2024 - Taylor & Francis
Background Biliary atresia (BA) is a severe neonatal progressive cholangiopathy of
unknown etiology. A timely Kasai portoenterostomy (KPE) improves survival of the native …

Planar cell polarity is crucial for proper morphogenesis of the bile ducts

FP Lemaigre - Journal of hepatology, 2024 - Elsevier
While apical-basal polarity refers to the distribution of cellular components between the
apical and basal poles of a cell, planar cell polarity (PCP) refers to the coordinated …

A beneficial response of fetal wound healing gone bad in the bile duct: the overarching cause of biliary atresia?

DC Trampert, U Beuers - Journal of Hepatology, 2024 - journal-of-hepatology.eu
Biliary atresia (BA) is a neonatal fibro-obliterative disease of the extrahepatic bile ducts.
Already in the first days to weeks after birth BA manifests in affected children with (sub) total …

Expression and clinical significance of MMP-13 in the liver of children with biliary atresia

XG Xiong, ZB Zheng, YM Liu, Y Liao, XD Qin, XH Zhao… - 2024 - researchsquare.com
Objective: This study aims to investigate the correlation between matrix metalloproteinase-
13 (MMP-13) expression in the liver of patients with biliary atresia and various factors …

[HTML][HTML] Forward genetics combined with unsupervised classifications identified zebrafish mutants affecting biliary system formation

DJ Singh, KM Tuscano, AL Ortega, M Dimri, K Tae… - Developmental …, 2024 - Elsevier
Impaired formation of the biliary network can lead to congenital cholestatic liver diseases;
however, the genes responsible for proper biliary system formation and maintenance have …