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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...

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Detaylı Bibliyografya
Yayımlandı:FEBS Lett
Asıl Yazarlar: Pande, Suchita, Bizilj, William, Guo, Hwai-Chen
Materyal Türü: Artigo
Dil:Inglês
Baskı/Yayın Bilgisi: 2018
Konular:
Online Erişim: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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