| Chapter title |
Glycosidases: Inborn Errors of Glycosphingolipid Catabolism
|
|---|---|
| Chapter number | 21 |
| Book title |
Glycobiology of the Nervous System
|
| Published in |
Advances in neurobiology, January 2014
|
| DOI | 10.1007/978-1-4939-1154-7_21 |
| Pubmed ID | |
| Book ISBNs |
978-1-4939-1153-0, 978-1-4939-1154-7
|
| Authors |
Hisashi Ashida, Yu-Teh Li, Ashida, Hisashi, Li, Yu-Teh |
| Abstract |
Glycosphingolipids (GSLs) are information-rich glycoconjugates that occur in nature mainly as constituents of biomembranes. Each GSL contains a complex carbohydrate chain linked to a ceramide moiety that anchors the molecule to biomembranes. In higher animals, catabolism of GSLs takes place in lysosomes where sugar chains in GSLs are hydrolyzed by exo-glycosidases to cleave a sugar residue from the non-reducing end of a sugar chain. Inborn errors of GSL-catabolism, collectively called sphingolipidoses or GSL-storage diseases, are caused by the deficiency of exo-glycosidases responsible for the degradation of the specific sugar residues at the non-reducing termini in GSLs. This chapter briefly discusses glycone, anomeric, linkage, and aglycone specificities of exo-glycosidases and some of the historical landmarks on their associations with the chemical pathology of the five best known sphingolipidoses: GM1 gangliosidosis, GM2 gangliosidosis (Tay-Sachs disease), Fabry disease, Gaucher disease, and Krabbe disease. |
Mendeley readers
Geographical breakdown
| Country | Count | As % |
|---|---|---|
| Unknown | 10 | 100% |
Demographic breakdown
| Readers by professional status | Count | As % |
|---|---|---|
| Researcher | 2 | 20% |
| Professor > Associate Professor | 2 | 20% |
| Other | 1 | 10% |
| Student > Master | 1 | 10% |
| Professor | 1 | 10% |
| Other | 2 | 20% |
| Unknown | 1 | 10% |
| Readers by discipline | Count | As % |
|---|---|---|
| Medicine and Dentistry | 3 | 30% |
| Biochemistry, Genetics and Molecular Biology | 2 | 20% |
| Agricultural and Biological Sciences | 2 | 20% |
| Economics, Econometrics and Finance | 1 | 10% |
| Unknown | 2 | 20% |