Issues in multi-organ transplantation of the liver with kidney or heart in polycystic liver-kidney disease or congenital heart disease: Current practices and …
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 …
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
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 …
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 …
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
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 …
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 …
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 …
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 …
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) …
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 …
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 …
cystic kidney disease phenotype. The association between these variants and liver cysts is …