Prominin‐1 Promotes Biliary Fibrosis Associated With Biliary Atresia

Jessica A. Zagory, Michael Fenlon, William Dietz, Menghan Zhao, Marie V. Nguyen, Pavin Trinh, Maeva Adoumie, Alex Park, Jiabo Xu, Elaa Mahdi, Alison Glazier, Nicolas Malkoff, Nirmala Mavila, Kasper S. Wang, in conjunction with the Childhood Liver Disease Research Network – 5 February 2019 – In patients with biliary atresia (BA), the extent of intrahepatic biliary fibrosis negatively correlates with successful surgical bypass of the congenital cholangiopathy as well as subsequent transplant‐free survival.

Outcomes After Listing for Liver Transplant in Patients With Acute‐on‐Chronic Liver Failure: The Multicenter North American Consortium for the Study of End‐Stage Liver Disease Experience

Jacqueline G. O’Leary, Jasmohan S. Bajaj, Puneeta Tandon, Scott W. Biggins, Florence Wong, Patrick S. Kamath, Guadalupe Garcia‐Tsao, Benedict Maliakkal, Jennifer Lai, Michael Fallon, Hugo E. Vargas, Paul Thuluvath, Ram Subramanian, Leroy R. Thacker, K. Rajender Reddy – 5 February 2019 – Acute‐on‐chronic liver failure (ACLF) characterized with ≥2 extrahepatic organ failures in cirrhosis carries a high mortality. Outcomes of patients listed for liver transplantation (LT) after ACLF and after LT are largely unknown.

Tumor Microenvironment Regulation by the Endoplasmic Reticulum Stress Transmission Mediator Golgi Protein 73 in Mice

Congwen Wei, Xiaoli Yang, Ning Liu, Jin Geng, Yanhong Tai, Zhenyu Sun, Gangwu Mei, Pengyu Zhou, Yumeng Peng, Chenbin Wang, Xiaoli Zhang, Pingping Zhang, Yunqi Geng, Yujie Wang, Xiaotong Zhang, Xin Liu, Yanhong Zhang, Feixiang Wu, Xiang He, Hui Zhong – 5 February 2019 – The unfolded protein response (UPR) signal in tumor cells activates UPR signaling in neighboring macrophages, which leads to tumor‐promoting inflammation by up‐regulating UPR target genes and proinflammatory cytokines.

Amelioration of Ductular Reaction by Stem Cell Derived Extracellular Vesicles in MDR2 Knockout Mice via Lethal‐7 microRNA

Kelly McDaniel, Nan Wu, Tianhao Zhou, Li Huang, Keisaku Sato, Julie Venter, Ludovica Ceci, Demeng Chen, Sugeily Ramos‐Lorenzo, Pietro Invernizzi, Francesca Bernuzzi, Chaodong Wu, Heather Francis, Shannon Glaser, Gianfranco Alpini, Fanyin Meng – 5 February 2019 – Cholangiopathies are diseases that affect cholangiocytes, the cells lining the biliary tract. Liver stem cells (LSCs) are able to differentiate into all cells of the liver and possibly influence the surrounding liver tissue by secretion of signaling molecules.

Improved Graft Survival After Liver Transplantation for Recipients With Hepatitis C Virus in the Direct‐Acting Antiviral Era

Thomas G. Cotter, Sonali Paul, Burhaneddin Sandıkçı, Thomas Couri, Adam S. Bodzin, Ester C. Little, Vinay Sundaram, Michael Charlton – 4 February 2019 – Highly effective direct‐acting antiviral (DAA) therapy has transformed outcomes of liver transplantation in hepatitis C virus (HCV) patients. We examined longer‐term outcomes in HCV‐positive recipients in the DAA era and analyzed the Scientific Registry of Transplant Recipients for primary adult, single‐organ, nonfulminant liver transplant recipients in the United States from January 1, 2008 to June 30, 2018.

Glial Cell Line–Derived Neurotrophic Factor Enhances Autophagic Flux in Mouse and Rat Hepatocytes and Protects Against Palmitate Lipotoxicity

Simon Musyoka Mwangi, Ge Li, Lan Ye, Yunshan Liu, Francois Reichardt, Samantha M. Yeligar, C. Michael Hart, Mark J. Czaja, Shanthi Srinivasan – 4 February 2019 – Glial cell line–derived neurotrophic factor (GDNF) is a protein that is required for the development and survival of enteric, sympathetic, and catecholaminergic neurons. We previously reported that GDNF is protective against high fat diet (HFD)‐induced hepatic steatosis in mice through suppression of hepatic expression of peroxisome proliferator activated receptor‐γ and genes encoding enzymes involved in de novo lipogenesis.

Extracellular Vesicle‐Based Therapeutic Targeting of β‐Catenin to Modulate Anticancer Immune Responses in Hepatocellular Cancer

Akiko Matsuda, Kaori Ishiguro, Irene K. Yan, Tushar Patel – 4 February 2019 – Hepatocellular carcinoma (HCC) is a leading cause of cancer‐related death worldwide. Although HCC can respond to immune checkpoint inhibitors, such as monoclonal antibodies against programmed death 1 (PD‐1), many patients fail to respond or develop secondary resistance. Activation of Wnt/β‐catenin signaling can contribute to immune evasion. Mutations in β‐catenin are among the most frequent mutations associated with HCC.

Surgical Aspects of Liver Transplantation and Domino Liver Transplantation in Maple Syrup Urine Disease: Analysis of 15 Donor‐Recipient Pairs

Uta Herden, Enke Grabhorn, René Santer, Jun Li, Silvio Nadalin, Xavier Rogiers, Marcus N. Scherer, Felix Braun, Jan Beime, Henning Lenhartz, Ania C. Muntau, Lutz Fischer – 3 February 2019 – Liver transplantation (LT) has been shown to be a feasible treatment in patients with severe forms of maple syrup urine disease (MSUD). Because of a sufficient extrahepatic enzyme activity in non‐MSUD individuals, the organ of MSUD patients can be used as a domino graft.

Liver Expression of a MiniATP7B Gene Results in Long‐Term Restoration of Copper Homeostasis in a Wilson Disease Model in Mice

Oihana Murillo, Daniel Moreno, Cristina Gazquez, Miren Barberia, Itziar Cenzano, Iñigo Navarro, Iker Uriarte, Victor Sebastian, Manuel Arruebo, Veronica Ferrer, Bernard Bénichou, Jean Philippe Combal, Jesus Prieto, Ruben Hernandez‐Alcoceba, Gloria Gonzalez Aseguinolaza – 1 February 2019 – Gene therapy with an adeno‐associated vector (AAV) serotype 8 encoding the human ATPase copper‐transporting beta polypeptide (ATP7B) complementary DNA (cDNA; AAV8‐ATP7B) is able to provide long‐term copper metabolism correction in 6‐week‐old male Wilson disease (WD) mice.

A Randomized, Controlled, Phase 2 Study of Maralixibat in the Treatment of Itching Associated With Primary Biliary Cholangitis

Marlyn J. Mayo, Paul J. Pockros, David Jones, Christopher L. Bowlus, Cynthia Levy, Imran Patanwala, Bruce Bacon, Velimir Luketic, Raj Vuppalanchi, Sharon Medendorp, Alejandro Dorenbaum, Ciara Kennedy, Patricia Novak, Joan Gu, George Apostol, Gideon M. Hirschfield – 1 February 2019 – Primary biliary cholangitis (PBC) is typically associated with elevated serum bile acid levels and pruritus, but pruritus is often refractory to treatment with existing therapies.

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