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Pathobiology of Hepatic Epithelia

Description: PROJECT NARRATIVE Polycystic liver disease (PLD) is an incurable genetic disorder characterized by progressive growth of hepatic cysts derived from bile duct cells (cholangiocytes). PLD causes considerable patient morbidity and there are no regulatory approved drugs for its treatment. Thus, with the goals of better understanding how cysts develop in PLD in order to identify effective therapies, this project explores the intersection between two cellular processes (defective autophagy and ciliogenesis) that underlie growth of liver cysts and are potential therapeutic targets.
Funding type: research
Funder scheme: R01
Start date: 1978-12-01
End date: 2024-03-31
Investigator: NICHOLAS F. LARUSSO
Institution: MAYO CLINIC ROCHESTER
Subfield: Genetics
Funded outputs count: 78
Funder award ID: R01DK024031
OpenAlex ID: G5361908396
Central role for cholangiocyte pathobiology in cholestatic liver diseases
2024 · Nidhi Jalan-Sakrikar, Maria Eugenia Guicciardi, et al. · Hepatology · 51
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Cholangiocyte ciliary defects induce sustained epidermal growth factor receptor signaling
2024 · Kishor Pant, Seth Richard, et al. · Hepatology · 12
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Regulator of G-protein signaling expression in human intestinal enteroendocrine cells and potential role in satiety hormone secretion in health and obesity
2024 · Alison N. McRae, Alexander L. Ticho, et al. · EBioMedicine · 8
Autophagy promotes hepatic cystogenesis in polycystic liver disease by depletion of cholangiocyte ciliogenic proteins
2021 · Anatoliy I. Masyuk, Tatyana V. Masyuk, et al. · Hepatology · 15
Long non-coding RNA ACTA2-AS1 promotes ductular reaction by interacting with the p300/ELK1 complex
2021 · Amaia Navarro-Corcuera, Tejasav S. Sehrawat, et al. · Journal of Hepatology · 56
Polycystic Liver Disease: Advances in Understanding and Treatment
2021 · Tatyana V. Masyuk, Anatoliy I. Masyuk, et al. · Annual Review of Pathology Mechanisms of Disease · 42
Induced Pluripotent Stem Cells From Subjects With Primary Sclerosing Cholangitis Develop a Senescence Phenotype Following Biliary Differentiation
2021 · Nidhi Jalan‐Sakrikar, Thiago M. De Assuncao, et al. · Hepatology Communications · 26
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Autophagy-mediated reduction of miR-345 contributes to hepatic cystogenesis in polycystic liver disease
2021 · Tatyana Masyuk, Anatoliy Masyuk, et al. · JHEP Reports · 8
Guidelines for the use and interpretation of assays for monitoring autophagy (4th edition) 1
2021 · Daniel J. Klionsky, Amal Kamal Abdel-Aziz, et al. · Autophagy · 2,955
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Targeting UBC9-mediated protein hyper-SUMOylation in cystic cholangiocytes halts polycystic liver disease in experimental models
2020 · Pui Y. Lee-Law, Paula Olaizola, et al. · Journal of Hepatology · 25
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Polarized human cholangiocytes release distinct populations of apical and basolateral small extracellular vesicles
2020 · Brian A. Davies, Leslie O. Morton, et al. · Molecular Biology of the Cell · 17
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Pansomatostatin Agonist Pasireotide Long-Acting Release for Patients with Autosomal Dominant Polycystic Kidney or Liver Disease with Severe Liver Involvement
2020 · Marie C. Hogan, Julie A. Chamberlin, et al. · Clinical Journal of the American Society of Nephrology · 41
Proteostasis disturbances and endoplasmic reticulum stress contribute to polycystic liver disease: New therapeutic targets
2020 · Alvaro Santos‐Laso, Laura Izquierdo‐Sanchez, et al. · Liver International · 25
Cholangiocyte pathobiology
2019 · Jesus M. Banales, Robert C. Huebert, et al. · Nature Reviews Gastroenterology & Hepatology · 542
MicroRNA (miR)‐433 and miR‐22 dysregulations induce histone‐deacetylase‐6 overexpression and ciliary loss in cholangiocarcinoma
2018 · Adrian P. Mansini, Maria J. Lorenzo Pisarello, et al. · Hepatology · 72
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Combination of a Histone Deacetylase 6 Inhibitor and a Somatostatin Receptor Agonist Synergistically Reduces Hepatorenal Cystogenesis in an Animal Model of Polycystic Liver Disease
2018 · Maria Lorenzo Pisarello, Tatyana V. Masyuk, et al. · American Journal Of Pathology · 22
Cholangiocytes in health and disease: From basic science to novel treatments
2018 · Jesus M. Banales, Marco Marzioni, et al. · Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease · 2
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Polycystic liver disease: The interplay of genes causative for hepatic and renal cystogenesis
2017 · Tatyana V. Masyuk, Anatoliy I. Masyuk, et al. · Hepatology · 15
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Cholangiocyte autophagy contributes to hepatic cystogenesis in polycystic liver disease and represents a potential therapeutic target
2017 · Anatoliy I. Masyuk, Tatyana V. Masyuk, et al. · Hepatology · 40
Development and characterization of cholangioids from normal and diseased human cholangiocytes as an in vitro model to study primary sclerosing cholangitis
2017 · Lorena Loarca, Thiago M De Assuncao, et al. · Laboratory Investigation · 53
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Pathobiology of biliary epithelia
2017 · Angela C. Cheung, Maria J. Lorenzo Pisarello, et al. · Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease · 47
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TGR5 contributes to hepatic cystogenesis in rodents with polycystic liver diseases through cyclic adenosine monophosphate/Gαs signaling
2017 · Tatyana V. Masyuk, Anatoliy I. Masyuk, et al. · Hepatology · 77
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Therapeutic Targets in Polycystic Liver Disease
2017 · Tatyana V. Masyuk, Anatoliy I. Masyuk, et al. · Current Drug Targets · 72
B-type natriuretic peptide overexpression ameliorates hepatorenal fibrocystic disease in a rat model of polycystic kidney disease
2017 · Sara J. Holditch, Claire A. Schreiber, et al. · Kidney International · 9
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Primary Sclerosing Cholangitis
2016 · Konstantinos N. Lazaridis, Nicholas F. LaRusso · New England Journal of Medicine · 539
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