Relevant bibliographies by topics / Congenital Hepatic fibrosis COLL1

Academic literature on the topic 'Congenital Hepatic fibrosis COLL1'

Author: Grafiati
Published: 9 March 2023
Last updated: 10 March 2023

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Journal articles on the topic "Congenital Hepatic fibrosis COLL1"

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Abdullah, Asaad M. A., and Hisham Nazer. "Congenital Hepatic Fibrosis." Annals of Saudi Medicine 15, no. 1 (January 1995): 82–83. http://dx.doi.org/10.5144/0256-4947.1995.82.

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Raskovic, Zorica, Biljana Vuletic, Zoran Igrutinovic, and Slavica Markovic. "Congenital hepatic fibrosis." Serbian Journal of Experimental and Clinical Research 14, no. 4 (2013): 175–79. http://dx.doi.org/10.5937/sjecr14-4339.

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De Vos, M., F. Barbier, and C. Cuvelier. "Congenital hepatic fibrosis." Journal of Hepatology 6, no. 2 (January 1988): 222–28. http://dx.doi.org/10.1016/s0168-8278(88)80036-9.

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Trizzino, Antonino, Piero Farruggia, Delia Russo, Paolo D??Angelo, Serena Tropia, Vincenzo Benigno, Giuseppe Tarantino, Vito Di Marco, and Maurizio Aric?? "Congenital Hepatic Fibrosis." Journal of Pediatric Hematology/Oncology 27, no. 10 (October 2005): 567–68. http://dx.doi.org/10.1097/01.mph.0000184577.46458.7e.

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Zlatković, Marija, Slaviša Ðuričić, and Pavle Plamenac. "Congenital hepatic fibrosis of heterotopic hepatic tissue." Pathology - Research and Practice 194, no. 7 (January 1998): 523–26. http://dx.doi.org/10.1016/s0344-0338(98)80123-4.

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DESMET, V. J. "What is congenital hepatic fibrosis?" Histopathology 20, no. 6 (April 3, 2007): 465–78. http://dx.doi.org/10.1111/j.1365-2559.1992.tb01031.x.

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Mankala, Vikas M., Jessica L. Davis, Chirag V. Patel, and Henry C. Lin. "Congenital Hepatic Fibrosis Presenting With Pancytopenia." JPGN Reports 2, no. 1 (January 13, 2021): e043. http://dx.doi.org/10.1097/pg9.0000000000000043.

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Yenice, Harun, Ziya Günal, and Sinan Erten. "A Case of Congenital Hepatic Fibrosis." Journal of Tepecik Education and Research Hospital 5, no. 1 (1995): 97–99. http://dx.doi.org/10.5222/terh.1995.08835.

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Zubair, Adeel S., Ali Metwaly, Justin M. Burns, and John A. Stauffer. "Intrahepatic cholangiocarcinoma with congenital hepatic fibrosis." International Journal of Hepatobiliary and Pancreatic Diseases 5 (2015): 103. http://dx.doi.org/10.5348/ijhpd-2015-43-cr-18.

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Wu, Wei Kelly, Ioannis A. Ziogas, Manhal Izzy, Anita K. Pai, Einar T. Hafberg, Lea K. Matsuoka, and Sophoclis P. Alexopoulos. "Liver transplantation for congenital hepatic fibrosis." Transplant International 34, no. 7 (May 30, 2021): 1281–92. http://dx.doi.org/10.1111/tri.13884.

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Dissertations / Theses on the topic "Congenital Hepatic fibrosis COLL1"

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LOCATELLI, LUIGI. "Expression of aVB6 integrin by Pkhd1-defective cholangiocytes links enhanced ductal secretion of Macrophage chemokines to progressive portal fibrosis in Congenital Hepatic Fibrosis." Doctoral thesis, Università degli Studi di Milano-Bicocca, 2013. http://hdl.handle.net/10281/41733.

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Abstract:
BACKGROUND AND AIMS: Congenital Hepatic Fibrosis (CHF) is caused by mutations in PKHD1, a gene encoding for fibrocystin, a protein of unknown function, expressed in cholangiocyte cilia and centromers. In CHF, biliary dysgenesis is accompanied by severe progressive portal fibrosis and portal hypertension. The mechanisms responsible for portal fibrosis in CHF are unclear. The αvβ6 integrin mediates local activation of TGFβ1 and is expressed by reactive cholangiocytes during cholestasis. To understand the mechanisms of fibrosis in CHF we studied the expression of αvβ6 integrin and its regulation in Pkhd1del4/del4 mice. METHODS: In Pkhd1del4/del4 mice we studied, at different ages (1-12 months): a) portal fibrosis (Sirius Red) and portal hypertension (spleen weight/body weight); b) αvβ6 mRNA and protein expression (RT-PCR, IHC); c) α-SMA and TGFβ1 mRNA expression (RT-PCR); d) portal inflammatory infiltrate (IHC for CD45 and FACS analysis of whole liver infiltrate); f) cytokines secretion from cultured monolayers of primary cholangiocytes (Luminex assay); g) cytokine effects on monocyte/macrophage proliferation (MTS assay) and migration (Boyden chamber); h) TGFβ1 and TNFα effects on β6 integrin mRNA expression by cultured cholangiocytes before and after inhibition of the TGFβ receptor type II (TGFβRII); i) TGFβ1 effects on collagen type I (COLL1) mRNA expression by cultured cholangiocytes. RESULTS: Pkhd1del4/del4 mice showed a progressive increase in αvβ6 integrin expression on biliary cyst epithelia. Expression of αvβ6 correlated with portal fibrosis (r=0.94, p<0.02) and with enrichment of a CD45+ve cell infiltrate in the portal space (r=0.97, p<0.01). Gene expression of TGFβ1 showed a similar age-dependent increase. FACS analysis showed that 50-75% of the CD45+ve cells were macrophages (CD45/CD11b/F4/80+ve). Cultured polarized Pkhd1del4/del4 cholangiocytes secreted from the basolateral side significantly increased amounts of CXCL1 and CXCL10 (p<0.05). Both cytokines were able to stimulate macrophage migration (p<0.05). Basal expression of β6 mRNA by cultured Pkhd1del4/del4 cholangiocytes (0.015±0.002 2^-dCt) was potently stimulated by the macrophage-derived cytokines TGFβ1 (0.017±0.002 2^-dCt, p<0.05) and TNFα (0.018±0.003 2^-dCt, p<0.05). Inhibition of TGFβRII completely blunted TGFβ1 (0.014±0.003 2^-dCt, p<0.05) but not TNFα effects (0.017±0.001 2^-dCt, p=ns) on β6 mRNA. COLL1 mRNA expression by cultured Pkhd1del4/del4 cholangiocytes (0.0009±0.0003 2^-dCt) was further and significantly increased after TGFβ1 stimulation (0.002±0.0005 2^-dCt, p<0.05). CONCLUSIONS: Pkhd1del4/del4 cholangiocytes possess increased basolateral secretory functions of chemokines (CXCL1, CXCL10) able to orchestrate macrophage homing to the peribiliary microenvironment. In turn, by releasing TGFβ1 and TNFα, macrophages up-regulate αvβ6 integrin in Pkhd1del4/del4 cholangiocytes. αvβ6 integrin activates latent TGFβ1, further increasing the fibrogenic properties of cholangiocytes.
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Book chapters on the topic "Congenital Hepatic fibrosis COLL1"

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Nazer, Dena, and Hisham M. Nazer. "Congenital Hepatic Fibrosis." In Textbook of Clinical Pediatrics, 2013–16. Berlin, Heidelberg: Springer Berlin Heidelberg, 2012. http://dx.doi.org/10.1007/978-3-642-02202-9_208.

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Reynolds, Telfer B. "Congenital Hepatic Fibrosis." In Portal Hypertension, 319–23. Tokyo: Springer Japan, 1991. http://dx.doi.org/10.1007/978-4-431-68361-2_25.

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Pavelka, Margit, and Jürgen Roth. "Congenital Hepatic Fibrosis." In Functional Ultrastructure, 220–21. Vienna: Springer Vienna, 2010. http://dx.doi.org/10.1007/978-3-211-99390-3_114.

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Arnon, Ronen, Henrietta Kotlus Rosenberg, and Frederick J. Suchy. "Caroli Disease, Caroli Syndrome, and Congenital Hepatic Fibrosis." In Fibrocystic Diseases of the Liver, 331–58. Totowa, NJ: Humana Press, 2010. http://dx.doi.org/10.1007/978-1-60327-524-8_14.

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Rock, Nathalie, Ino Kanavaki, and Valérie McLin. "Congenital Hepatic Fibrosis, Caroli’s Disease, and Other Fibrocystic Liver Diseases." In Textbook of Pediatric Gastroenterology, Hepatology and Nutrition, 647–61. Cham: Springer International Publishing, 2015. http://dx.doi.org/10.1007/978-3-319-17169-2_57.

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Rock, N. M., I. Kanavaki, and V. A. McLin. "Congenital Hepatic Fibrosis, Caroli’s Disease, and Other Fibrocystic Liver Diseases." In Textbook of Pediatric Gastroenterology, Hepatology and Nutrition, 791–806. Cham: Springer International Publishing, 2021. http://dx.doi.org/10.1007/978-3-030-80068-0_60.

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LOOMES, KATHLEEN M., and MATTHEW J. RYAN. "Congenital Hepatic Fibrosis." In Pediatric Gastroenterology, 253–60. Elsevier, 2008. http://dx.doi.org/10.1016/b978-0-323-03280-3.50036-3.

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"Congenital Hepatic Fibrosis." In Diagnostic Imaging: Gastrointestinal, 590–93. Elsevier, 2015. http://dx.doi.org/10.1016/b978-0-323-37755-3.50188-4.

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"Congenital Hepatic Fibrosis." In Diagnostic Pathology: Hepatobiliary and Pancreas, 44–45. Elsevier, 2017. http://dx.doi.org/10.1016/b978-0-323-44307-4.50021-x.

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"Congenital Hepatic Fibrosis." In High-Yield Imaging: Gastrointestinal, 907–8. Elsevier, 2010. http://dx.doi.org/10.1016/b978-1-4160-5544-0.00359-7.

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Conference papers on the topic "Congenital Hepatic fibrosis COLL1"

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Christoph, Sandra, Guido Gerken, Hartmut Schmidt, and Alisan Kahraman. "A patient with congenital hepatic fibrosis, after living-donor liver transplantation, developed a systemic IgG4-related disease following infection with SARS-CoV-2." In 38. Jahrestagung der Deutsche Arbeitsgemeinschaft zum Studium der Leber. Georg Thieme Verlag, 2022. http://dx.doi.org/10.1055/s-0041-1740797.

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