Issues in multi-organ transplantation of the liver with kidney or heart in polycystic liver-kidney disease or congenital heart disease: Current practices and …

T Taner, MB Hilscher, CR Broda, JPH Drenth - Journal of hepatology, 2023 - Elsevier
Solid organ transplantation has become an integral part of the management of patients with
end-stage diseases of the kidney, liver, heart and lungs. Most procedures occur in isolation …

Opportunities and considerations for studying liver disease with microphysiological systems on a chip

AE Otumala, DJ Hellen, CA Luna, P Delgado… - Lab on a Chip, 2023 - pubs.rsc.org
Advances in microsystem engineering have enabled the development of highly controlled
models of the liver that better recapitulate the unique in vivo biological conditions. In just a …

A SEC61A1 variant is associated with autosomal dominant polycystic liver disease

B Schlevogt, V Schlieper, J Krader, R Schröter… - Liver …, 2023 - Wiley Online Library
Abstract Background and Aims Autosomal dominant polycystic liver and kidney disease is a
spectrum of hereditary diseases, which display disturbed function of primary cilia leading to …

Glis2 is an early effector of polycystin signaling and a target for therapy in polycystic kidney disease

C Zhang, M Rehman, X Tian, SLC Pei, J Gu… - Nature …, 2024 - nature.com
Mouse models of autosomal dominant polycystic kidney disease (ADPKD) show that intact
primary cilia are required for cyst growth following the inactivation of polycystin-1. The …

Autophagy promotes hepatic cystogenesis in polycystic liver disease by depletion of cholangiocyte ciliogenic proteins

AI Masyuk, TV Masyuk, CE Trussoni, NE Pirius… - Hepatology, 2022 - journals.lww.com
Requirement of RORα for maintenance and antitumor immunity o... : Hepatology Requirement of
RORα for maintenance and antitumor immunity of liver‐resident natural killer cells/ILC1s …

Heterozygosity of ALG9 in association with autosomal dominant polycystic liver disease

MM Boerrigter, R Duijzer, RHM Te Morsche… - Genes, 2023 - mdpi.com
α-1, 2-mannosyltransferase (ALG9) germline variants are linked to autosomal dominant
polycystic kidney disease (ADPKD). Many individuals affected with ADPKD possess …

Multi-omics profiling of cholangiocytes reveals sex-specific chromatin state dynamics during hepatic cystogenesis in polycystic liver disease

R Ji, J Chen, Y Xie, X Dou, B Qing, Z Liu, Y Lu… - Journal of …, 2023 - Elsevier
Background & Aims Cholangiocytes transit from quiescence to hyperproliferation during
cystogenesis in polycystic liver disease (PLD), the severity of which displays prominent sex …

Wall Tension and Tubular Resistance in Kidney Cystic Conditions

M Della Corte, D Viggiano - Biomedicines, 2023 - mdpi.com
The progressive formation of single or multiple cysts accompanies several renal diseases.
Specifically,(i) genetic forms, such as adult dominant polycystic kidney disease (ADPKD) …

Comparative analysis of SEC61A1 mutant R236C in two patient-derived cellular platforms

M Weiand, V Sandfort, O Nadzemova… - Scientific Reports, 2024 - nature.com
SEC61A1 encodes a central protein of the mammalian translocon and dysfunction results in
severe disease. Recently, mutation R236C was identified in patients having autosomal …

Novel α-1, 3-Glucosyltransferase Variants and Their Broad Clinical Polycystic Liver Disease Spectrum

MM Boerrigter, RHM Te Morsche, H Venselaar… - Genes, 2023 - mdpi.com
Protein-truncating variants in α-1, 3-glucosyltransferase (ALG8) are a risk factor for a mild
cystic kidney disease phenotype. The association between these variants and liver cysts is …