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Synthesis and characterization of a new fluorogenic substrate for alpha-galactosidase

Alpha-galactosidase A hydrolyzes the terminal alpha-galactosyl moieties from glycolipids and glycoproteins in lysosomes. Mutations in α-galactosidase cause lysosomal accumulation of the glycosphingolipid, globotriaosylceramide, which leads to Fabry disease. Small-molecule chaperones that bind to mut...

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Bibliografiske detaljer
Main Authors: Shi, Zhen-Dan, Motabar, Omid, Goldin, Ehud, Liu, Ke, Southall, Noel, Sidransky, Ellen, Austin, Christopher P., Griffiths, Gary L., Zheng, Wei
Format: Artigo
Sprog:Inglês
Udgivet: 2009
Fag:
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC2756488/
https://ncbi.nlm.nih.gov/pubmed/19521690
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1007/s00216-009-2879-5
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