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Glycopeptide storage in skin fibroblasts cultured from a patient with alpha-mannosidase deficiency.
Patients with mannosidosis, an inherited deficiency of lysosomal alpha-mannosidase, accumulate large amounts of mannose-rich oligosaccharides (the "core" of the carbohydrate units of many glocoproteins) in brain and liver and excrete these partial degradation products in their urine. A pro...
Uloženo v:
| Hlavní autoři: | , , |
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| Médium: | Artigo |
| Jazyk: | Inglês |
| Vydáno: |
1975
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| Témata: | |
| On-line přístup: | https://ncbi.nlm.nih.gov/pmc/articles/PMC301920/ https://ncbi.nlm.nih.gov/pubmed/125765 |
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