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https://www.readbyqxmd.com/read/29162437/mouse-model-of-alagille-syndrome-and-mechanisms-of-jagged1-missense-mutations
#1
Emma R Andersson, Indira V Chivukula, Simona Hankeova, Marika Sjöqvist, Yat Long Tsoi, Daniel Ramsköld, Jan Masek, Aiman Elmansuri, Anita Hoogendoorn, Elenae Vazquez, Helena Storvall, Julie Netušilová, Meritxell Huch, Björn Fischler, Ewa Ellis, Adriana Contreras, Antal Nemeth, Kenneth C Chien, Hans Clevers, Rickard Sandberg, Vitezslav Bryja, Urban Lendahl
BACKGROUND & AIMS: Alagille syndrome is a genetic disorder characterized by cholestasis, ocular abnormalities, characteristic facial features, heart defects, and vertebral malformations. Most cases are associated with mutations in JAGGED1 (JAG1), which encodes a Notch ligand, although it is not clear how these contribute to disease development. We aimed to develop a mouse model of Alagille syndrome to elucidate these mechanisms. METHODS: Mice with a missense mutation (H268Q) in Jag1 (Jag1(+)/(Ndr) mice) were outbred to a C3H/C57bl6 background to generate a mouse model for Alagille syndrome (Jag1(Ndr/Ndr) mice)...
November 18, 2017: Gastroenterology
https://www.readbyqxmd.com/read/29158418/a-novel-pkhd1-mutation-interacts-with-the-nonobese-1diabetic-genetic-background-to-cause-autoimmune-cholangitis
#2
Wenting Huang, Daniel B Rainbow, Yuehong Wu, David Adams, Pranavkumar Shivakumar, Leah Kottyan, Rebekah Karns, Bruce Aronow, Jorge Bezerra, M Eric Gershwin, Laurence B Peterson, Linda S Wicker, William M Ridgway
We previously reported that NOD.c3c4 mice develop spontaneous autoimmune biliary disease (ABD) with anti-mitochondrial Abs, histopathological lesions, and autoimmune T lymphocytes similar to human primary biliary cholangitis. In this article, we demonstrate that ABD in NOD.c3c4 and related NOD ABD strains is caused by a chromosome 1 region that includes a novel mutation in polycystic kidney and hepatic disease 1 (Pkhd1). We show that a long terminal repeat element inserted into intron 35 exposes an alternative polyadenylation site, resulting in a truncated Pkhd1 transcript...
November 20, 2017: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/29154966/determinants-of-fibrosis-progression-and-regression-in-nash
#3
REVIEW
Detlef Schuppan, Rambabu Surabattula, Xiao Yu Wang
Cirrhosis has become the major liver related clinical endpoint in nonalcoholic steatohepatitis. However, progression to cirrhosis is less predictable in NASH compared to other chronic liver diseases. This is due to the complex and multifactorial etiology of NASH, which is determined by life style and nutrition, multiple genetic and epigenetic factors, and a prominent role of hepatic and extrahepatic comorbidities. Thus modest changes in these cofactors can also induce fibrosis regression, at least in precirrhotic patients...
November 14, 2017: Journal of Hepatology
https://www.readbyqxmd.com/read/29154784/tgf-%C3%AE-1-signaling-regulates-mouse-hepatic-stellate-cell-differentiation-via-the-jagged1-notch-pathway
#4
Yasen Aimaiti, Xin Jin, Wei Wang, Zixin Chen, Dewei Li
AIMS: We tested whether transforming growth factor β1 (TGF-β1) signaling plays an important role in hepatic stellate cell differentiation fate and investigated the role of Jagged1/Notch in this process. MATERIALS AND METHODS: TGF-β1 was overexpressed and transforming growth factor receptor 1 (TGF-β-R1) was knocked down by a lentiviral vector in mouse hepatic stellate cells (mHSCs). Transfection efficiency was assessed with immunofluorescence, quantitative reverse-transcriptase polymerase chain reaction (qRT-PCR) and western blotting...
November 14, 2017: Life Sciences
https://www.readbyqxmd.com/read/29149928/rheumatic-manifestations-in-autoimmune-liver-disease
#5
REVIEW
Carlo Selmi, Elena Generali, Merrill Eric Gershwin
Autoimmune liver diseases coexist with rheumatic disorders in approximately 30% of cases and may also share pathogenic mechanisms. Autoimmune liver diseases result from an immune-mediated injury of different tissues, with autoimmune hepatitis (AIH) targeting hepatocytes, and primary biliary cholangitis (PBC) and primary sclerosing cholangitis targeting cholangiocytes. Sjogren syndrome is diagnosed in 7% of AIH cases and serologic autoimmunity profiles are a common laboratory abnormality, particularly in the case of serum antimitochondrial (PBC) or anti-liver kidney microsomal antibodies (AIH)...
February 2018: Rheumatic Diseases Clinics of North America
https://www.readbyqxmd.com/read/29140564/%C3%AE-catenin-and-il-1%C3%AE-dependent-cxcl10-production-drives-progression-of-disease-in-a-mouse-model-of-congenital-hepatic-fibrosis
#6
Eleanna Kaffe, Romina Fiorotto, Francesca Pellegrino, Valeria Mariotti, Mariangela Amenduni, Massimiliano Cadamuro, Luca Fabris, Mario Strazzabosco, Carlo Spirli
Congenital Hepatic Fibrosis (CHF), a genetic disease caused by mutations in the PKHD1 gene, encoding for the protein fibrocystin (FPC), is characterized by biliary dysgenesis, progressive portal fibrosis, and by a PKA-mediated activating phosphorylation of β-Catenin at Ser675. Biliary structures of Pkhd1(del4/del4) mice, a mouse model of CHF, secrete CXCL10 a chemokine able to recruit macrophages. The aim of this study is to clarify whether CXCL10 plays a pathogenetic role in disease progression in CHF/CD and to understand the mechanisms leading to increased CXCL10 secretion...
November 15, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/29116012/connexin-32-and-connexin-43-are-involved-in-lineage-restriction-of-hepatic-progenitor-cells-to-hepatocytes
#7
Haiyun Pei, Chao Zhai, Huilin Li, Fang Yan, Jinhua Qin, Hongfeng Yuan, Rui Zhang, Shuyong Wang, Wencheng Zhang, Mingyang Chang, Yunfang Wang, Xuetao Pei
BACKGROUND: Bi-potential hepatic progenitor cells can give rise to both hepatocytes and cholangiocytes, which is the last phase and critical juncture in terms of sequentially hepatic lineage restriction from any kind of stem cells. If their differentiation can be controlled, it might access to functional hepatocytes to develop pharmaceutical and biotechnology industries as well as cell therapies for end-stage liver diseases. METHODS: In this study, we investigated the influence of Cx32 and Cx43 on hepatocyte differentiation of WB-F344 cells by in vitro gain and loss of function analyses...
November 7, 2017: Stem Cell Research & Therapy
https://www.readbyqxmd.com/read/29100373/chronically-high-level-of-tgfb1a-induction-causes-both-hepatocellular-carcinoma-and-cholangiocarcinoma-via-a-dominant-erk-pathway-in-zebrafish
#8
Chuan Yan, Qiqi Yang, Han-Ming Shen, Jan M Spitsbergen, Zhiyuan Gong
Liver cancers including both hepatocellular carcinoma (HCC) and cholangiocarcinoma (CCA) have increased steadily with the prevalence of non-alcoholic steatohepatitis (NASH), but the underlying mechanism for the transition from NASH to liver cancers remains unclear. Here we first employed diet-induced NASH zebrafish and found that elevated level of satiety hormone, leptin, induced overexpression of tgfb1. Then we developed tgfb1a transgenic zebrafish for inducible, hepatocyte-specific expression. Interestingly, chronically high tgfb1a induction in hepatocytes could concurrently drive both HCC and CCA...
September 29, 2017: Oncotarget
https://www.readbyqxmd.com/read/29097260/advances-in-the-generation-of-bioengineered-bile-ducts
#9
REVIEW
Alexander W Justin, Kourosh Saeb-Parsy, Athina E Markaki, Ludovic Vallier, Fotios Sampaziotis
The generation of bioengineered biliary tissue could contribute to the management of some of the most impactful cholangiopathies associated with liver transplantation, such as biliary atresia or ischemic cholangiopathy. Recent advances in tissue engineering and in vitro cholangiocyte culture have made the achievement of this goal possible. Here we provide an overview of these developments and review the progress towards the generation and transplantation of bioengineered bile ducts.
October 30, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/29095436/rna-sequencing-based-comparative-analysis-of-human-hepatic-progenitor-cells-and-their-niche-from-alcoholic-steatohepatitis-livers
#10
An Ceulemans, Stefaan Verhulst, Matthias Van Haele, Olivier Govaere, Juan-Jose Ventura, Leo A van Grunsven, Tania Roskams
Hepatic progenitor cells (HPCs) are small cells with a relative large oval nucleus and a scanty cytoplasm situated in the canals of Hering that express markers of (immature) hepatocytes and cholangiocytes. HPCs are present in large numbers in alcoholic steatohepatitis (ASH), one of the leading causes of chronic liver disease. To date, the mechanisms responsible for proliferation and differentiation of human HPCs are still poorly understood and the role of HPCs in ASH development is unknown. In this study, we aimed to characterise human HPCs and their interactions with other cells through comparison, on both protein and RNA level, of HPC-enriched cell populations from adult human liver tissue using different isolation methods...
November 2, 2017: Cell Death & Disease
https://www.readbyqxmd.com/read/29080340/bile-acids-in-cholestasis-and-its-treatment
#11
Juan Pablo Arab, Daniel Cabrera, Marco Arrese
Bile acids (BA) are key molecules in generating bile flow, which is an essential function of the liver. In the last decades there have been great advances in the understanding of the role of a number of specific transport proteins present at the sinusoidal and canalicular membrane domains of hepatocytes and cholangiocytes in generating and maintaining bile flow. Also, a clearer understanding on how BA regulate their own synthesis and the expression and/or function of transporters has been reached. This new knowledge has helped to better delineate the pathophysiology of cholestasis and the adaptive responses of hepatocytes to cholestatic liver injury as well as of the mechanisms of injury of biliary epithelia...
October 28, 2017: Annals of Hepatology
https://www.readbyqxmd.com/read/29067165/recent-advances-in-understanding-cholangiocarcinoma
#12
REVIEW
Lindsey Kennedy, Laura Hargrove, Jennifer Demieville, Nicole Francis, Rowan Seils, Sara Villamaria, Heather Francis
Cholangiocarcinoma (CCA) is an aggressive malignancy that arises from damaged epithelial cells, cholangiocytes, and possibly de-differentiated hepatocytes. CCA has a poor overall survival rate and limited therapeutic options. Based on this data, it is imperative that new diagnostic and therapeutic interventions be developed. Recent work has attempted to understand the pathological mechanisms driving CCA progression. Specifically, recent publications have delved into the role of cancer stem cells (CSCs), mesenchymal stem cells (MSCs), and microRNAs (miRNAs) during CCA pathology...
2017: F1000Research
https://www.readbyqxmd.com/read/29052842/shear-stress-upregulates-regeneration-related-immediate-early-genes-in-liver-progenitors-in-3d-ecm-like-microenvironments
#13
Kenichiro Nishii, Erik Brodin, Taylor Renshaw, Rachael Weesner, Emma Moran, Shay Soker, Jessica L Sparks
The role of fluid stresses in activating the hepatic stem/progenitor cell regenerative response is not well understood. This study hypothesized that immediate early genes (IEGs) with known links to liver regeneration will be upregulated in liver progenitor cells (LPCs) exposed to in vitro shear stresses on the order of those produced from elevated interstitial flow after partial hepatectomy. The objectives were: (1) to develop a shear flow chamber for application of fluid stress to LPCs in 3D culture; and (2) to determine the effects of fluid stress on IEG expression in LPCs...
October 20, 2017: Journal of Cellular Physiology
https://www.readbyqxmd.com/read/29023826/cftr-actin-g-as-a-master-regulator-of-cholangiocyte-function
#14
EDITORIAL
Andrew P Feranchak
No abstract text is available yet for this article.
October 10, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/29023824/cholangiocyte-autophagy-contributes-to-hepatic-cystogenesis-in-polycystic-liver-disease-and-represents-a-potential-therapeutic-target
#15
Anatoliy I Masyuk, Tatyana V Masyuk, Maria J Lorenzo Pisarello, Jingyi Francess Ding, Lorena Loarca, Bing Q Huang, Nicholas F LaRusso
Polycystic liver disease (PLD) is a group of genetic disorders with limited treatment and significant morbidity. Hepatic cysts arise from cholangiocytes exhibiting a hyperproliferative phenotype. Considering that hyperproliferation of many cell types is associated with alterations in autophagy, we hypothesized that autophagy is altered in PLD cholangiocytes, contributes to hepatic cystogenesis, and might represent a potential therapeutic target. We employed Functional Pathway Cluster Analysis (FPCA) and NextGen Sequencing (NGS), transmission electron microscopy, immunofluorescence confocal microscopy, and western blotting to assess autophagy in human and rodent PLD cholangiocytes...
October 10, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/29017397/liver-scaffolds-support-survival-and-metabolic-function-of-multilineage-neonatal-allogenic-cells
#16
Wessam Hassanein, Arielle Cimeno, Avraham Werdesheim, Bryan Buckingham, Joshua Harrison, Mehmet C Uluer, Ali Khalifeh, Carlos Rivera-Pratts, Stephen Klepfer, Jhade D Woodall, Urmil Dhru, Elliot Bromberg, Dawn Parsell, Cinthia Drachenberg, Rolf N Barth, John C LaMattina
Organ scaffold bioengineering is currently limited by the inability to effectively repopulate the scaffold with appropriately distributed functional cells. We examined the feasibility of a decellularized liver scaffold to support the growth and function of multilineage allogenic cells derived from either adult or neonatal liver cells. Cell slurries from neonatal and adult rat livers containing hepatocytes, cholangiocytes, and endothelial cells were introduced into decellularized adult rat liver scaffolds via the bile duct...
October 10, 2017: Tissue Engineering. Part A
https://www.readbyqxmd.com/read/28994423/cholangiocarcinoma-evolving-concepts-and-therapeutic-strategies
#17
REVIEW
Sumera Rizvi, Shahid A Khan, Christopher L Hallemeier, Robin K Kelley, Gregory J Gores
Cholangiocarcinoma is a disease entity comprising diverse epithelial tumours with features of cholangiocyte differentiation: cholangiocarcinomas are categorized according to anatomical location as intrahepatic (iCCA), perihilar (pCCA), or distal (dCCA). Each subtype has a distinct epidemiology, biology, prognosis, and strategy for clinical management. The incidence of cholangiocarcinoma, particularly iCCA, has increased globally over the past few decades. Surgical resection remains the mainstay of potentially curative treatment for all three disease subtypes, whereas liver transplantation after neoadjuvant chemoradiation is restricted to a subset of patients with early stage pCCA...
October 10, 2017: Nature Reviews. Clinical Oncology
https://www.readbyqxmd.com/read/28973524/liver-cyst-gene-knockout-in-cholangiocytes-inhibits-cilium-formation-and-wnt-signaling
#18
Edgar S Wills, René H M Te Morsche, Jeroen van Reeuwijk, Nicola Horn, Iris Geomini, Liyanne F M van de Laarschot, Dorus A Mans, Marius Ueffing, Karsten Boldt, Joost P H Drenth, Ronald Roepman
Mutations in the PRKCSH, SEC63 and LRP5 genes cause autosomal dominant polycystic liver disease (ADPLD). The proteins products of PRKCSH (alias GIIB) and SEC63 function in protein quality control and processing in the endoplasmic reticulum (ER), while LRP5 is implicated in Wnt/β-catenin signaling. To identify common denominators in the PLD pathogenesis, we mapped the PLD interactome by affinity proteomics, employing both HEK293T cells and H69 cholangiocytes. Identification of known complex members, such as glucosidase IIA (GIIA) for PRKCSH, and SEC61A1 and SEC61B for SEC63, confirmed the specificity of the analysis...
November 1, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28965883/nuclear-receptor-fxr-bile-acids-and-liver-damage-introducing-the-progressive-familial-intrahepatic-cholestasis-with-fxr-mutations
#19
REVIEW
Marica Cariello, Elena Piccinin, Oihane Garcia-Irigoyen, Carlo Sabbà, Antonio Moschetta
The nuclear receptor farnesoid X receptor (FXR) is the master regulator of bile acids (BAs) homeostasis since it transcriptionally drives modulation of BA synthesis, influx, efflux, and detoxification along the enterohepatic axis. Due to its crucial role, FXR alterations are involved in the progression of a plethora of BAs associated inflammatory disorders in the liver and in the gut. The involvement of the FXR pathway in cholestasis development and management has been elucidated so far with a direct role of FXR activating therapy in this condition...
September 29, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28962898/role-of-the-bicarbonate-responsive-soluble-adenylyl-cyclase-in-cholangiocyte-apoptosis-in-primary-biliary-cholangitis-a-new-hypothesis
#20
REVIEW
Jung-Chin Chang, Simei Go, Arthur J Verhoeven, Ulrich Beuers, Ronald P J Oude Elferink
Primary biliary cholangitis (PBC) is a chronic fibrosing cholangiopathy characterized by an autoimmune stereotype and defective biliary bicarbonate secretion due to down-regulation of anion exchanger 2 (AE2). Despite the autoimmune features, immunosuppressants are ineffective while two bile acid-based therapies (ursodeoxycholic acid and obeticholic acid) have been shown to improve biochemical and histological features of cholestasis and long-term prognosis. However, the etiology and pathogenesis of PBC is largely unknown...
September 26, 2017: Biochimica et Biophysica Acta
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