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- W2007509470 abstract "inhibited in Idua−/− recipients, and there is a similar engraftment defect in Idua−/− recipients under non-myeloablative conditioning regimens. Idua−/− bone marrow HS is present in significant excess, particularly in extracellular matrix, cell surface and golgi, with significant increases in 2-O-, 6-Oand N-sulphation. Finally we show that excess HS, and particularly HS rich in 2-O-sulphate modifications, which is the most prevalent modification in MPS IH, functionally inhibits haematopoietic cell migration. Conclusion: These data provide novel insight into the mechanism behind historical graft failure after HSCT in MPS I and identify a functional role for highly sulphated HS in inhibiting CXCL12 mediated haematopoietic cell migration in the Idua−/− mouse model of MPS I." @default.
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- W2007509470 date "2013-02-01" @default.
- W2007509470 modified "2023-09-27" @default.
- W2007509470 title "Visual attention in children with Morquio syndrome" @default.
- W2007509470 doi "https://doi.org/10.1016/j.ymgme.2012.11.040" @default.
- W2007509470 hasPublicationYear "2013" @default.
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