Lactosylceramide in lysosomal storage disorders: a comparative immunohistochemical and biochemical study
Language English Country Germany Media print-electronic
Document type Comparative Study, Journal Article, Research Support, Non-U.S. Gov't
- MeSH
- Biomarkers analysis MeSH
- Antigens, CD analysis metabolism MeSH
- Chromatography, Thin Layer methods MeSH
- Child MeSH
- Adult MeSH
- Histiocytes chemistry metabolism pathology MeSH
- Immunohistochemistry methods MeSH
- Liver chemistry metabolism pathology MeSH
- Lactosylceramides analysis metabolism MeSH
- Humans MeSH
- Lysosomal Storage Diseases classification metabolism pathology MeSH
- Macrophages chemistry metabolism pathology MeSH
- Cerebral Cortex chemistry metabolism pathology MeSH
- Neurons chemistry metabolism pathology MeSH
- Spleen chemistry metabolism pathology MeSH
- Check Tag
- Child MeSH
- Adult MeSH
- Humans MeSH
- Publication type
- Journal Article MeSH
- Research Support, Non-U.S. Gov't MeSH
- Comparative Study MeSH
- Names of Substances
- Biomarkers MeSH
- Antigens, CD MeSH
- CDw17 antigen MeSH Browser
- Lactosylceramides MeSH
Immunohistochemical studies of the presence of lactosylceramide (LacCer) in lysosomal storage disorders (LSDs) were done using anti-LacCer monoclonal antibody of the CDw 17 type (clone MG-2). No sign of an association between LacCer and the lysosomal system in normal cells was observed, except for histiocytes active in phagocytosis. A comparative study of a group of LSDs showed a general tendency for LacCer to increase in storage cells in Niemann-Pick disease type C (NPC), and types A and B, GM1 gangliosidosis, acid lipase deficiency, glycogen storage disease type II and mucopolysaccharidoses. LacCer accumulated in storage cells despite normal activity of relevant lysosomal degrading enzymes. The accumulation of LacCer displayed variability within storage cell populations, and was mostly expressed in neurons in NPC. An absence of the increase in LacCer in storage cells above control levels was seen in neuronal ceroid lipofuscinoses (neurons and cardiocytes) and in Fabry disease. Gaucher and Krabbe cells showed significantly lower levels, or even the absence, of LacCer compared with control macrophages. Results of immunohistochemistry were corroborated by semiquantitative lipid thin-layer chromatography (TLC). It is suggested that different associations of LacCer with the lysosomal storage process may reflect differences in glycosphingolipid turnover induced by the storage-compromised lysosomal/endosomal system.
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