- Abstract:
-
Paediatric neurodegenerative diseases are frequently caused by inborn errors in glycosphingolipid (GSL) catabolism and are collectively termed the glycosphingolipidoses. GSL catabolism occurs in the lysosome and a defect in an enzyme involved in GSL degradation leads to the lysosomal storage of its substrate(s). GSLs are abundantly expressed in the central nervous system (CNS) and the disorders frequently have a progressive neurodegenerative course. Our understanding of pathogenesis in these ...
Expand abstract - Publication status:
- Published
- Publisher copy:
- 10.1046/j.1365-2990.2002.00422.x
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- Journal:
- Neuropathology and applied neurobiology
- Volume:
- 28
- Issue:
- 5
- Pages:
- 343-357
- Publication date:
- 2002-10-05
- DOI:
- EISSN:
-
1365-2990
- ISSN:
-
0305-1846
- URN:
-
uuid:0c5f5348-57e6-4492-831d-ab0f2a4cd84e
- Source identifiers:
-
100957
- Local pid:
- pubs:100957
- Language:
- English
- Keywords:
- Copyright date:
- 2002
Journal article
Glycosphingolipid lysosomal storage diseases: therapy and pathogenesis.
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