In silicoanalyses of essential interactions of iminosugars with the Hex A active site and evaluation of their pharmacological chaperone effects for Tay–Sachs disease-Reference-Cited by-同舟云学术

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In silicoanalyses of essential interactions of iminosugars with the Hex A active site and evaluation of their pharmacological chaperone effects for Tay–Sachs disease

Published:2017 Issue:44 Volume:15 Page:9297-9304
ISSN:1477-0520
Container-title:Organic & Biomolecular Chemistry
language:en
Short-container-title:Org. Biomol. Chem.

Author:

Kato Atsushi¹²³⁴^ORCID,Nakagome Izumi⁵⁶⁷⁴,Nakagawa Shinpei¹²³⁴,Kinami Kyoko¹²³⁴,Adachi Isao¹²³⁴,Jenkinson Sarah F.⁸⁹¹⁰¹¹¹²,Désiré Jérôme¹³¹⁴¹⁵¹⁶¹⁷,Blériot Yves¹³¹⁴¹⁵¹⁶¹⁷^ORCID,Nash Robert J.¹⁸¹⁹²⁰²¹¹²,Fleet George W. J.⁸⁹¹⁰¹¹¹²^ORCID,Hirono Shuichi⁵⁶⁷⁴

Affiliation:

1. Department of Hospital Pharmacy

2. University of Toyama

3. Toyama 930-0194

4. Japan

5. School of Pharmaceutical Sciences

6. Kitasato University

7. Tokyo 108-8641

8. Chemistry Research Laboratory

9. Department of Chemistry

10. University of Oxford

11. Oxford

12. UK

13. Equipe Synthèse Organique

14. Groupe Glycochimie

15. IC2MP

16. UMR CNRS 7285

17. Université de Poitiers

18. Institute of Biological

19. Environmental and Rural Sciences/Phytoquest Limited

20. Plas Gogerddan

21. Aberystwyth, Ceredigion

Abstract

DMDP amide restored Hex A activity in the G269S Tay–Sachs patient cells up to 43% of the wild type.

Publisher

Royal Society of Chemistry (RSC)

Subject

Organic Chemistry,Physical and Theoretical Chemistry,Biochemistry

Link

http://pubs.rsc.org/en/content/articlepdf/2017/OB/C7OB02281F

Reference28 articles.

1. Assignment of β-hexosaminidase A α-subunit to human chromosomal region 15q23→q24

2. Xmnl RFLP at 5q13 detected by a 0.49 kb Xmn I fragment of human hexosaminidase (HEXB) cDNA

3. 17. Naturally occurring mutations in GM2 gangliosidosis: A compendium

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5. Structural variation of the 3-acetamido-4,5,6-trihydroxyazepane iminosugar through epimerization and C-alkylation leads to low micromolar HexAB and NagZ inhibitors;Organic & Biomolecular Chemistry;2022

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