Biliary Atresia–emerging diagnostic and therapy opportunities

U Lendahl, VCH Lui, PHY Chung, PKH Tam - EBioMedicine, 2021 - thelancet.com
Biliary Atresia is a devastating pediatric cholangiopathy affecting the bile ducts of the liver. In
this review, we describe recent progress in the understanding of liver development with a …

[HTML][HTML] Jagged-mediated development and disease: Mechanistic insights and therapeutic implications for Alagille syndrome

J Mašek, ER Andersson - Current Opinion in Cell Biology, 2024 - Elsevier
Notch signaling controls multiple aspects of embryonic development and adult homeostasis.
Alagille syndrome is usually caused by a single mutation in the jagged canonical Notch …

Spatially segregated defects and IGF1‐responsiveness of hilar and peripheral biliary organoids from a model of Alagille syndrome

A Iqbal, N Van Hul, L Belicova, AA Corbat… - Liver …, 2024 - Wiley Online Library
Abstract Background & Aims Alagille syndrome (ALGS) manifests with peripheral
intrahepatic bile duct (IHBD) paucity, which can spontaneously resolve. In a model for ALGS …

[HTML][HTML] Mesoscale visualization of three-dimensional microvascular architecture and immunocyte distribution in intact mouse liver lobes

Z Liu, M Xu, S Huang, Q Pan, C Liu, F Zeng, Z Fan… - Theranostics, 2022 - ncbi.nlm.nih.gov
Rational: The complex vascular architecture and diverse immune cells of the liver are critical
for maintaining liver homeostasis. However, quantification of the network of liver vasculature …

Jag1 insufficiency alters liver fibrosis via T cell and hepatocyte differentiation defects

J Mašek, I Filipovic, N Van Hul, L Belicová… - EMBO Molecular …, 2024 - embopress.org
Fibrosis contributes to tissue repair, but excessive fibrosis disrupts organ function. Alagille
syndrome (ALGS, caused by mutations in JAGGED1) results in liver disease and …

Axon guidance genes control hepatic artery development

L Gannoun, C De Schrevel, M Belle… - …, 2023 - journals.biologists.com
Earlier data on liver development demonstrated that morphogenesis of the bile duct, portal
mesenchyme and hepatic artery is interdependent, yet how this interdependency is …

Sex differences and risk factors for bleeding in Alagille syndrome

S Hankeova, N Van Hul, J Laznovsky… - EMBO Molecular …, 2022 - embopress.org
Spontaneous bleeds are a leading cause of death in the pediatric JAG1‐related liver
disease Alagille syndrome (ALGS). We asked whether there are sex differences in bleeding …

Three-dimensional structure of liver vessels and spatial distribution of hepatic immune cells

M Xu, Z Liu, X Li, X Wang, X Yuan, C Han… - Journal of Innovative …, 2023 - World Scientific
As the largest internal organ of the human body, the liver has an extremely complex vascular
network and multiple types of immune cells. It plays an important role in blood circulation …

BiliQML: A supervised machine-learning model to quantify biliary forms from digitized whole-slide liver histopathological images

DJ Hellen, ME Fay, DH Lee… - American Journal …, 2024 - journals.physiology.org
The progress of research focused on cholangiocytes and the biliary tree during development
and following injury is hindered by limited available quantitative methodologies. Current …

[HTML][HTML] The Notch Signaling Pathway: Mechanistic Insights in Health and Disease

Y Meng, Z Bo, X Feng, X Yang, PA Handford - Engineering, 2024 - Elsevier
The Notch signaling pathway is evolutionarily conserved across metazoan species and
plays key roles in many physiological processes. The Notch receptor is activated by two …