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Biochemical and Structural Insights into an Allelic Variant Causing the Lysosomal Storage Disorder Aspartylglucosaminuria
Aspartylglucosaminuria (AGU) is a lysosomal storage disorder caused by defects of the hydrolase glycosylasparaginase (GA). Previously, we showed that a Canadian AGU mutation disrupts an obligatory intra-molecular autoprocessing with the enzyme trapped as an inactive precursor. Here, we report bioche...
Αποθηκεύτηκε σε:
| Τόπος έκδοσης: | FEBS Lett |
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| Κύριοι συγγραφείς: | , , |
| Μορφή: | Artigo |
| Γλώσσα: | Inglês |
| Έκδοση: |
2018
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| Θέματα: | |
| Διαθέσιμο Online: | https://ncbi.nlm.nih.gov/pmc/articles/PMC6119092/ https://ncbi.nlm.nih.gov/pubmed/29993127 https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1002/1873-3468.13190 |
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