Stereodivergent synthesis of right- and left-handed iminoxylitol heterodimers and monomers. Study of their impact on β-glucocerebrosidase activity-Reference-Cited by-同舟云学术

Stereodivergent synthesis of right- and left-handed iminoxylitol heterodimers and monomers. Study of their impact on β-glucocerebrosidase activity

Published:2017 Issue:17 Volume:15 Page:3681-3705
ISSN:1477-0520
Container-title:Organic & Biomolecular Chemistry
language:en
Short-container-title:Org. Biomol. Chem.

Author:

Stauffert Fabien¹²³⁴⁵,Serra-Vinardell Jenny⁶⁷⁸⁹¹⁰,Gómez-Grau Marta⁶⁷⁸⁹¹⁰,Michelakakis Helen¹¹¹²¹³¹⁴¹⁵,Mavridou Irene¹¹¹²¹³¹⁴¹⁵,Grinberg Daniel⁶⁷⁸⁹¹⁰^ORCID,Vilageliu Lluïsa⁶⁷⁸⁹¹⁰,Casas Josefina¹⁶¹⁷¹⁸¹⁹²⁰^ORCID,Bodlenner Anne¹²³⁴⁵,Delgado Antonio¹⁶¹⁷¹⁸¹⁹²⁰^ORCID,Compain Philippe¹²³⁴⁵^ORCID

Affiliation:

1. Laboratoire de Synthèse Organique et Molécules Bioactives (SYBIO)

2. Université de Strasbourg/CNRS (UMR 7509)

3. Ecole Européenne de Chimie

4. Polymères et Matériaux (ECPM)

5. 67087 Strasbourg

6. Departament de Genètica

7. Microbiologia i Estadística

8. Universitat de Barcelona (UB)

9. IBUB, IRSJD

10. CIBER de Enfermedades Raras (CIBERER)

11. Department of Enzymology and Cellular Function

12. Institute of Child Health

13. Athens

14. 11527

15. Greece

16. Research Unit on BioActive Molecules (RUBAM)

17. Departament de Química Biomèdica

18. Institut de Química Avançada de Catalunya (IQAC-CSIC)

19. 08034 Barcelona

20. Spain

Abstract

Unprecedented Janus-faced iminosugars act as pharmacological chaperones for the treatment of Gaucher disease.

Funder

Ministerio de Ciencia e Innovación

Centre National de la Recherche Scientifique

Université de Strasbourg

Publisher

Royal Society of Chemistry (RSC)

Subject

Organic Chemistry,Physical and Theoretical Chemistry,Biochemistry

Link

http://pubs.rsc.org/en/content/articlepdf/2017/OB/C7OB00443E

Reference65 articles.

1. E. Beutler and G. A.Grabowski, in The Metabolic and Molecular Bases of Inherited Disease, ed. C. Scriver, A. L. Beaudet, W. S. Sly and D. Valle, McGraw-Hill, New York, 8th edn, 2001, pp. 3635–3668

2. Phenotype, diagnosis, and treatment of Gaucher's disease

3. Biosynthesis of two components of human acid α-glucosidase

4. Intracellular transport of acid ?-glucosidase and lysosome-associated membrane proteins is affected in Gaucher's disease (G202R mutation)

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