Výsledky vyhledávání - "Viuff, Agnete"

Molecular Mechanisms of Disease Pathogenesis Differ in Krabbe Disease Variants

Autor: Spratley, Samantha J, Hill, Chris H, Viuff, Agnete H, Edgar, James R, Skjødt, Karsten, Deane, Janet E

Publikováno v: Traffic (Copenhagen, Denmark)
Spratley, S J, Hill, C H, Viuff, A H, Edgar, J R, Skjødt, K & Deane, J E 2016, ' Molecular Mechanisms of Disease Pathogenesis Differ in Krabbe Disease Variants ', Traffic (Malden), vol. 17, no. 8, pp. 908-922 . https://doi.org/10.1111/tra.12404
Spratley, S J, Hill, C H, Viuff, A H, Edgar, J R, Skjødt, K & Deane, J E 2016, ' Molecular Mechanisms of Disease Pathogenesis Differ in Krabbe Disease Variants ', Traffic, vol. 17, no. 8, pp. 908-922 . https://doi.org/10.1111/tra.12404

Krabbe disease is a severe, fatal neurodegenerative disorder caused by defects in the lysosomal enzyme galactocerebrosidase (GALC). The correct targeting of GALC to the lysosome is essential for the degradation of glycosphingolipids including the pri

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=pmid_dedup__::744bff627c234b133de9e06616f53262
http://europepmc.org/articles/PMC4949656

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Akademický článek

The Bicyclic Form of galacto-Noeurostegine Is a Potent Inhibitor of β‑Galactocerebrosidase.

Autor: Viuff, Agnete, Salamone, Stéphane, McLoughlin, Joseph, Deane, Janet E., Jensen, Henrik H.

Publikováno v: ACS Medicinal Chemistry Letters; 1/14/2021, Vol. 12 Issue 1, p56-59, 4p

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Iminosugars and isoiminosugars as pharmacological chaperones for Krabbe disease - a SAR study:and synthesis and evaluation of novel nortropanes as glycosidase inhibitors

Autor: Viuff, Agnete

Publikováno v: Viuff, A 2015, Iminosugars and isoiminosugars as pharmacological chaperones for Krabbe disease-a SAR study : and synthesis and evaluation of novel nortropanes as glycosidase inhibitors .

The overall theme of this thesis is the use of iminosugars and isoiminosugars as inhibitors or chaperones for glycosidases. Glycosidases are a group of enzymes with a large variety of biological roles, and the ability to modulate their activity is, t

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=pure_au_____::d212add6a9431cb003e415abe2619b7f
https://pure.au.dk/portal/da/publications/iminosugars-and-isoiminosugars-as-pharmacological-chaperones-for-krabbe-disease--a-sar-study(a1033db4-2a81-42f5-bcbc-e65e25e68402).html

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Azasugar inhibitors as pharmacological chaperones for Krabbe disease

Autor: Hill, Chris H., Viuff, Agnete, Spratley, Samantha J., Salamone, Stephane, Christensen, Stig H., Read, Randy J., Moriarty, Nigel W., Jensen, Henrik Helligsø, Deane, Janet E.

Publikováno v: Hill, C H, Viuff, A, Spratley, S J, Salamone, S, Christensen, S H, Read, R J, Moriarty, N W, Jensen, H H & Deane, J E 2015, ' Azasugar inhibitors as pharmacological chaperones for Krabbe disease ', Chemical Science, vol. 6, no. 5, pp. 3075-3086 . https://doi.org/10.1039/c5sc00754b

Krabbe disease is a devastating neurodegenerative disorder characterized by rapid demyelination of nerve fibers. This disease is caused by defects in the lysosomal enzyme beta-galactocerebrosidase (GALC), which hydrolyzes the terminal galactose from

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=pure_au_____::cd4ffd8cb3a190daa214cfff0c89aaca
https://pure.au.dk/portal/da/publications/azasugar-inhibitors-as-pharmacological-chaperones-for-krabbe-disease(e45e01b3-24cd-4702-8079-1b8fa19da53e).html

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