Leptomeningeal lipid storage patterns in Fabry disease
| Autor: | H. Christomanou, Klaus Harzer, B. Kustermann-Kuhn, Milan Elleder |
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| Rok vydání: | 1994 |
| Předmět: |
Male
Pathology medicine.medical_specialty Cell type Central nervous system Lipid metabolism Biology Lipid Metabolism medicine.disease Lipid storage Fabry disease Phenotype Epithelium Pathophysiology Pathology and Forensic Medicine Cellular and Molecular Neuroscience medicine.anatomical_structure medicine Fabry Disease Humans Neurology (clinical) Arachnoid Aged |
| Zdroj: | Acta Neuropathologica. 88:579-582 |
| ISSN: | 1432-0533 0001-6322 |
| Popis: | We found two patterns of leptomeningeal storage that reflect two basic visceral storage patterns in Fabry disease. (i) A generalized-type leptomeningeal storage pattern, affecting all main leptomeningeal cell types (external arachnoideal epithelium, fibroblasts, vessel wall elements), was a consistent finding in three cases of classical generalized visceral phenotype. (ii) A localized leptomeningeal storage pattern was expressed, to a high degree, solely in the external arachnoidal epithelium; this pattern was found in one case with the variant visceral-restricted-type storage (confined to the cardiocytes). Thus, the external arachnoidal epithelium may be particurlarly susceptible to Fabry lipid storage, probably caused by a distinctly larger sustained lysosomal lipid load as compared to other cell types. |
| Databáze: | OpenAIRE |
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