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Original Article

Ultrastructure of the Liver in the Cerebrohepatorenal Syndrome of Zellweger

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Pages 135-144 | Accepted 08 Apr 1983, Published online: 10 Jul 2009

Keep up to date with the latest research on this topic with citation updates for this article.

Read on this site (2)

Mikako Warren, Gary Mierau, Eric P. Wartchow, Hiroyuki Shimada & Shoji Yano. (2018) Histologic and ultrastructural features in early and advanced phases of Zellweger spectrum disorder (infantile Refsum disease). Ultrastructural Pathology 42:3, pages 220-227.
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Derek A. Applegarth & James E. Dimmick. (1985) Annotated Bibliography: Adrenoleukodystrophy, Cerebrohepatorenal Syndrome (Zellweger Syndrome), and Peroxisomes. Pediatric Pathology 3:2-4, pages 377-378.
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Articles from other publishers (54)

Alberto Quaglia, Eve A. Roberts & Michael Torbenson. 2024. MacSween's Pathology of the Liver. MacSween's Pathology of the Liver 122 294 .
Lijun Chi, Dorothy Lee, Sharon Leung, Guanlan Hu, Bijun Wen, Paul Delgado-Olguin, Miluska Vissa, Ren Li, John H. Brumell, Peter K. Kim & Robert H. J. Bandsma. (2023) Loss of functional peroxisomes leads to increased mitochondrial biogenesis and reduced autophagy that preserve mitochondrial function. Cellular and Molecular Life Sciences 80:7.
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Michael Torbenson, Sanjay Kakar & Kay Washington. 2022. Non-Neoplastic Diseases of the Liver. Non-Neoplastic Diseases of the Liver 299 351 .
Paul A. Watkins, Lingxiao Chen & Nancy Braverman. 2021. Liver Disease in Children. Liver Disease in Children 671 697 .
Jay H. Lefkowitch. 2021. Scheuer's Liver Biopsy Interpretation. Scheuer's Liver Biopsy Interpretation 410 430 .
Tanja Eberhart, Miriam J. Schönenberger, Katharina M. Walter, Khanichi N. Charles, Phyllis L. Faust & Werner J. Kovacs. (2020) Peroxisome-Deficiency and HIF-2α Signaling Are Negative Regulators of Ketohexokinase Expression. Frontiers in Cell and Developmental Biology 8.
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Kevin Berendse, Bart G. P. Koot, Femke C. C. Klouwer, Marc Engelen, Frank Roels, Miangela M. Lacle, Peter G. J. Nikkels, Joanne Verheij & Bwee Tien Poll‐The. (2019) Hepatic symptoms and histology in 13 patients with a Zellweger spectrum disorder. Journal of Inherited Metabolic Disease 42:5, pages 955-965.
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James E. Heubi, Kenneth D.R. Setchell & Kevin E. Bove. (2018) Long-Term Cholic Acid Therapy in Zellweger Spectrum Disorders. Case Reports in Gastroenterology 12:2, pages 360-372.
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Abhijit Babaji Shinde, Ritesh Kumar Baboota, Simone Denis, Ursula Loizides-Mangold, Annelies Peeters, Marc Espeel, Ana Rita Malheiro, Howard Riezman, Stefan Vinckier, Frédéric M. Vaz, Pedro Brites, Sacha Ferdinandusse, Paul P. Van Veldhoven & Myriam Baes. (2018) Mitochondrial disruption in peroxisome deficient cells is hepatocyte selective but is not mediated by common hepatic peroxisomal metabolites. Mitochondrion 39, pages 51-59.
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Alberto Quaglia, Eve A. Roberts & Michael Torbenson. 2018. Macsween's Pathology of the Liver. Macsween's Pathology of the Liver 111 274 .
Myriam Baes & Paul P. Van Veldhoven. (2016) Hepatic dysfunction in peroxisomal disorders. Biochimica et Biophysica Acta (BBA) - Molecular Cell Research 1863:5, pages 956-970.
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Jay H. Lefkowitch. 2016. Scheuer's Liver Biopsy Interpretation. Scheuer's Liver Biopsy Interpretation 383 403 .
Richard J. Thompson, Bernard C. Portmann & Eve A. Roberts. 2012. MacSween's Pathology of the Liver. MacSween's Pathology of the Liver 157 259 .
Annelies Peeters, Peter Fraisl, Sjoerd van den Berg, Emiel Ver Loren van Themaat, Antoine Van Kampen, Mark H. Rider, Hiroshi Takemori, Ko Willems van Dijk, Paul P. Van Veldhoven, Peter Carmeliet & Myriam Baes. (2011) Carbohydrate Metabolism Is Perturbed in Peroxisome-deficient Hepatocytes Due to Mitochondrial Dysfunction, AMP-activated Protein Kinase (AMPK) Activation, and Peroxisome Proliferator-activated Receptor γ Coactivator 1α (PGC-1α) Suppression. Journal of Biological Chemistry 286:49, pages 42162-42179.
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Jay H. Lefkowitch. 2010. Scheuer's Liver Biopsy Interpretation. Scheuer's Liver Biopsy Interpretation 361 381 .
Sven Thoms, Sabine Grønborg & Jutta Gärtner. (2009) Organelle interplay in peroxisomal disorders. Trends in Molecular Medicine 15:7, pages 293-302.
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Shikha S Sundaram, Kevin E Bove, Mark A Lovell & Ronald J Sokol. (2008) Mechanisms of Disease: inborn errors of bile acid synthesis. Nature Clinical Practice Gastroenterology & Hepatology 5:8, pages 456-468.
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Megan H. Keane, Henk Overmars, Thomas M. Wikander, Sacha Ferdinandusse, Marinus Duran, Ronald J. A. Wanders & Phyllis L. Faust. (2007) Bile acid treatment alters hepatic disease and bile acid transport in peroxisome-deficient PEX2 Zellweger mice . Hepatology 45:4, pages 982-997.
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Ruud Dirkx, Ilse Vanhorebeek, Katrin Martens, Arno Schad, Markus Grabenbauer, Dariush Fahimi, Peter Declercq, Paul P. Van Veldhoven & Myriam Baes. (2005) Absence of peroxisomes in mouse hepatocytes causes mitochondrial and ER abnormalities. Hepatology 41:4, pages 868-878.
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Kevin E. Bove, James E. Heubi, William F. Balistreri & Kenneth D.R. Setchell. (2019) Bile Acid Synthetic Defects and Liver Disease: A Comprehensive Review. Pediatric and Developmental Pathology 7:4, pages 315-334.
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Gareth P. Jevon & James E. Dimmick. 2004. Pathology of Pediatric Gastrointestinal and Liver Disease. Pathology of Pediatric Gastrointestinal and Liver Disease 270 299 .
Marianne Depreter, Marc Espeel & Frank Roels. (2003) Human peroxisomal disorders. Microscopy Research and Technique 61:2, pages 203-223.
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Eveline Baumgart, Ilse Vanhorebeek, Markus Grabenbauer, Marcel Borgers, Peter E. Declercq, H. Dariush Fahimi & Myriam Baes. (2001) Mitochondrial Alterations Caused by Defective Peroxisomal Biogenesis in a Mouse Model for Zellweger Syndrome (PEX5 Knockout Mouse). The American Journal of Pathology 159:4, pages 1477-1494.
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H. Denk, H. P. Dienes, J. Düllmann, H.-P. Fischer, O. Klinge, W. Lierse, K.-H. Meyer zum Büschenfelde, U. Pfeifer, K. H. Preisegger, G. Ramadori, A. Tannapfel, Ch. Wittekind, U. Wulfhekel & H. ZhouK. H. Preisegger & H. Denk. 2000. Pathologie der Leber und Gallenwege. Pathologie der Leber und Gallenwege 239 316 .
William F. Balistreri. (1999) INBORN ERRORS OF BILE ACID BIOSYNTHESIS AND TRANSPORT. Gastroenterology Clinics of North America 28:1, pages 145-172.
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F. Roels, B. De Prest & G. De Pestel. (1995) Liver and chorion cytochemistry. Journal of Inherited Metabolic Disease 18:S1, pages 155-171.
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F. Roels, B. De Prest & G. De Pestel. 1995. Diagnosis of human peroxisomal disorders. Diagnosis of human peroxisomal disorders 155 171 .
Sadaki Yokota & Kohtaro Asayama. (1993) Proliferation of myocardial peroxisomes in experimental rat diabetes: a biochemical and immunocytochemical study. Virchows Archiv B Cell Pathology Including Molecular Pathology 63:1, pages 43-49.
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Hugo W. Moser. 1993. Advances in Human Genetics 21. Advances in Human Genetics 21 1 106 .
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F. Roels, M. Espeel & D. De Craemer. (1991) Liver pathology and immunocytochemistry in congenital peroxisomal diseases: a review. Journal of Inherited Metabolic Disease 14:6, pages 853-875.
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R.J.A. Wanders, E. Boltshauser, B. Steinmann, M.A. Spycher, R.B.H. Schutgens, H. van den Bosch & J.M. Tager. (1990) Infantile phytanic acid storage disease, a disorder of peroxisome biogenesis: a case report. Journal of the Neurological Sciences 98:1, pages 1-11.
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J. L. Hughes, A. Poulos, E. Robertson, C. W. Chow, L. J. Sheffield, J. Christodoulou & R. F. Carter. (1990) Pathology of hepatic peroxisomes and mitochondria in patients with peroxisomal disorders. Virchows Archiv A Pathological Anatomy and Histopathology 416:3, pages 255-264.
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Alf Poulos. (1989) Lipid metabolism in Zellweger's syndrome. Progress in Lipid Research 28:1, pages 35-51.
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I. Kerckaert, K. P. Dingemans, H. S. A. Heymans, J. Vamecq & F. Roels. (1987) Polarizing inclusions in some organs of children with congenital peroxisomal diseases (Zellweger's, Refsum's, chondrodysplasia punctata (rhizomelic form), X‐linked adrenoleukodystrophy). Journal of Inherited Metabolic Disease 11:4, pages 372-386.
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G. M. Small, M. J. Santos, T. Imanaka, A. Poulos, D. M. Danks, H. W. Moser & P. B. Lazarow. (1987) Peroxisomal integral membrane proteins in livers of patients with Zellweger syndrome, infantile Refsum's disease and X‐linked adrenoleukodystrophy. Journal of Inherited Metabolic Disease 11:4, pages 358-371.
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M J Santos, T Imanaka, H Shio & P B Lazarow. (1988) Peroxisomal integral membrane proteins in control and Zellweger fibroblasts.. Journal of Biological Chemistry 263:21, pages 10502-10509.
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A. Torvik, S. Torp, B.F. Kase, J. Ek, O. Skjeldal & O. Stokke. (1988) Infantile Refsum's disease: A generalized peroxisomal disorder. Journal of the Neurological Sciences 85:1, pages 39-53.
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Erich Kaiser & Robert Kramar. (1988) Clinical biochemistry of peroxisomal disorders. Clinica Chimica Acta 173:1, pages 57-80.
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R. B. H. Schutgens, R. J. A. Wanders, H. S. A. Heymans, A. W. Schram, J. M. Tager, G. Schrakamp & H. van den Bosch. (1987) Zellweger syndrome: Biochemical procedures in diagnosis, prevention and treatment. Journal of Inherited Metabolic Disease 10:S1, pages 33-45.
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William R. Treem, Camillus A. Witzleben, David A. Piccoli, Charles A. Stanley, Daniel E. Hale, Paul M. Coates & John B. Watkins. (1986) Medium-chain and long-chain acyl CoA dehydrogenase deficiency: Clinical, pathologic and ultrastructural differentiation from Reye's syndrome. Hepatology 6:6, pages 1270-1278.
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John M. Opitz. (1985) The Zellweger syndrome: Book review and bibliography. The cerebro-hepato-renal syndrome of Zellweger. L.C.P. Govaerts. Nijmegen: Stichting Studentenpers, 1984, 195 pp.. American Journal of Medical Genetics 22:2, pages 419-426.
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