Phosphorylation prevents polyglucosan transport in Lafora disease
| Autor: | Jean Marie Girard, Hannes Lohi, Julie Turnbull, Berge A. Minassian, Arman Draginov, Cátia M. Teixeira, Scellig S D Stone, Afra H. Wang, Paul W. Frankland, Christophe Blaszykowski, Xiao Chu Zhao, Cameron Ackerley, Peixiang Wang |
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| Rok vydání: | 2012 |
| Předmět: |
Nervous system
Biological Transport Active Biology Lafora disease Limited access chemistry.chemical_compound Glycogen phosphorylase Mice medicine Animals Phosphorylation Clinical/Scientific Notes Glucans chemistry.chemical_classification Mice Knockout Glycogen medicine.disease Cell biology Enzyme medicine.anatomical_structure nervous system Biochemistry chemistry Lafora Disease Cell bodies Neurology (clinical) |
| Zdroj: | Neurology. 79(1) |
| ISSN: | 1526-632X |
| Popis: | Neuronal distal axons have limited access to glucose, their myelin sheaths preventing direct interface with blood, and their axoplasms being at great distances from their cell bodies. Glycogen, the mammalian glucose store, is characterized by extreme branching, which allows packing 55,000 glucoses/glycogen, and maintaining solubility. In neurons, glycogen phosphorylase, the glycogen-digesting enzyme, is located in distal axons where it can generate large amounts of glucose from glycogen.1 |
| Databáze: | OpenAIRE |
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