Ultrastructural and Biochemical Liver Analyses in Fabry's Disease
| العنوان: | Ultrastructural and Biochemical Liver Analyses in Fabry's Disease |
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| المؤلفون: | S. G. M. Meuwissen, Joseph M. Tager, Kurt P. Dingemans, Anneke Strijland, Bert C. M. Ooms |
| المصدر: | Hepatology. 2:263S-268S |
| بيانات النشر: | Ovid Technologies (Wolters Kluwer Health), 2007. |
| سنة النشر: | 2007 |
| مصطلحات موضوعية: | Male, Pathology, medicine.medical_specialty, Ceramide, Hepatology, medicine.diagnostic_test, Biopsy, Middle Aged, Biology, Fabry's disease, Glycosphingolipids, Microscopy, Electron, chemistry.chemical_compound, Liver, chemistry, Liver biopsy, Organelle, medicine, Ultrastructure, Fabry Disease, Humans, lipids (amino acids, peptides, and proteins) |
| الوصف: | Ultrastructural and biochemical analyses were made of liver biopsy material from a patient with longstanding Fabry's disease. Both hepatocytes as well as periportal macrophages showed lipid accumulations consisting of amorphous material as well as stacks of lamellar leaflets. Lipid inclusions in periportal macrophages were much larger than in hepatocytes. Furthermore, small round spheres were found exclusively in periportal macrophages. The biochemical analysis showed an increased content of ceramide −3, with only minor elevated concentrations of other glycosphin-golipids. The almost normal hepatic architecture and the presence of well-preserved hepatocytic organelles are in agreement with the observation that liver involvement in Fabry's disease has only minor clinical significance. |
| تدمد: | 1527-3350 0270-9139 |
| URL الوصول: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::800befb2edd212df39f578fa6f49e61d https://doi.org/10.1002/hep.1840020214 |
| حقوق: | CLOSED |
| رقم الأكسشن: | edsair.doi.dedup.....800befb2edd212df39f578fa6f49e61d |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 15273350 02709139 |
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