Wnt–β-catenin signalling in liver development, health and disease

MJ Perugorria, P Olaizola, I Labiano… - Nature reviews …, 2019 - nature.com
The canonical Wnt–β-catenin pathway is a complex, evolutionarily conserved signalling
mechanism that regulates fundamental physiological and pathological processes. Wnt–β …

Genetics, pathobiology and therapeutic opportunities of polycystic liver disease

P Olaizola, PM Rodrigues… - Nature Reviews …, 2022 - nature.com
Polycystic liver diseases (PLDs) are inherited genetic disorders characterized by
progressive development of intrahepatic, fluid-filled biliary cysts (more than ten), which …

Pathobiology of inherited biliary diseases: a roadmap to understand acquired liver diseases

L Fabris, R Fiorotto, C Spirli, M Cadamuro… - Nature Reviews …, 2019 - nature.com
Bile duct epithelial cells, also known as cholangiocytes, regulate the composition of bile and
its flow. Acquired, congenital and genetic dysfunctions in these cells give rise to a set of …

Polycystic liver disease: advances in understanding and treatment

TV Masyuk, AI Masyuk… - Annual Review of …, 2022 - annualreviews.org
Polycystic liver disease (PLD) is a group of genetic disorders characterized by progressive
development of cholangiocyte-derived fluid-filled hepatic cysts. PLD is the most common …

[HTML][HTML] Transient Receptor Potential Vanilloid (TRPV4) channel inhibition: A novel promising approach for the treatment of lung diseases

M Kumar, MK Zaman, S Das, D Goyary… - Biomedicine & …, 2023 - Elsevier
Research on transient receptor potential vanilloid-4 (TRPV4) can provide a promising
potential therapeutic target in the development of novel medicines for lung disorders. TRPV4 …

[HTML][HTML] Polycystic liver disease: classification, diagnosis, treatment process, and clinical management

ZY Zhang, ZM Wang, Y Huang - World journal of hepatology, 2020 - ncbi.nlm.nih.gov
Polycystic liver disease (PLD) is a rare hereditary disease that independently exists in
isolated PLD, or as an accompanying symptom of autosomal dominant polycystic kidney …

Apoptosis and autophagy in polycystic kidney disease (PKD)

KL Nowak, CL Edelstein - Cellular signalling, 2020 - Elsevier
Apoptosis in the cystic epithelium is observed in most rodent models of polycystic kidney
disease (PKD) and in human autosomal dominant PKD (ADPKD). Apoptosis inhibition …

Polycystic liver disease: pathophysiology, diagnosis and treatment

LF Norcia, EM Watanabe… - Hepatic Medicine …, 2022 - Taylor & Francis
Polycystic liver disease (PLD) is a clinical condition characterized by the presence of more
than 10 cysts in the liver. It is a rare disease Of genetic etiology that presents as an isolated …

HDAC6-dependent ciliophagy is involved in ciliary loss and cholangiocarcinoma growth in human cells and murine models

E Peixoto, S Jin, K Thelen, A Biswas… - American Journal …, 2020 - journals.physiology.org
Reduced ciliary expression is reported in several tumors, including cholangiocarcinoma
(CCA). We previously showed primary cilia have tumor suppressor characteristics, and …

Proteostasis disturbances and endoplasmic reticulum stress contribute to polycystic liver disease: new therapeutic targets

A Santos‐Laso, L Izquierdo‐Sanchez… - Liver …, 2020 - Wiley Online Library
Abstract Background & Aims Polycystic liver diseases (PLDs) are genetic disorders
characterized by progressive development of multiple biliary cysts. Recently, novel PLD …