Jump to ContentJump to Main Navigation
Show Summary Details
More options …

Biological Chemistry

Editor-in-Chief: Brüne, Bernhard

Editorial Board Member: Buchner, Johannes / Lei, Ming / Ludwig, Stephan / Sies, Helmut / Thomas, Douglas D. / Turk, Boris / Wittinghofer, Alfred

12 Issues per year

IMPACT FACTOR 2016: 3.273

CiteScore 2016: 3.01

SCImago Journal Rank (SJR) 2016: 1.679
Source Normalized Impact per Paper (SNIP) 2016: 0.800

See all formats and pricing
More options …
Volume 389, Issue 11 (Nov 2008)


Acid β-glucosidase: insights from structural analysis and relevance to Gaucher disease therapy

Yaacov Kacher / Boris Brumshtein
  • 2Department of Neurobiology, Weizmann Institute of Science, Rehovot 76100, Israel and Department of Structural Biology, Weizmann Institute of Science, Rehovot 76100, Israel
  • Other articles by this author:
  • De Gruyter OnlineGoogle Scholar
/ Swetlana Boldin-Adamsky
  • Present address: QBI Enterprises Ltd., Weizmann Science Park, P.O. Box 4071, Nes Ziona 70400, Israel
    3Department of Biological Chemistry, Weizmann Institute of Science, Rehovot 76100, Israel
  • Other articles by this author:
  • De Gruyter OnlineGoogle Scholar
/ Lilly Toker / Alla Shainskaya
  • 5Biological Mass Spectrometry Unit, Biological Services, Weizmann Institute of Science, Rehovot 76100, Israel
  • Other articles by this author:
  • De Gruyter OnlineGoogle Scholar
/ Israel Silman / Joel L. Sussman / Anthony H. Futerman
Published Online: 2008-09-11 | DOI: https://doi.org/10.1515/BC.2008.163


In mammalian cells, glucosylceramide (GlcCer), the simplest glycosphingolipid, is hydrolyzed by the lysosomal enzyme acid β-glucosidase (GlcCerase). In the human metabolic disorder Gaucher disease, GlcCerase activity is significantly decreased owing to one of approximately 200 mutations in the GlcCerase gene. The most common therapy for Gaucher disease is enzyme replacement therapy (ERT), in which patients are given intravenous injections of recombinant human GlcCerase; the Genzyme product Cerezyme® has been used clinically for more than 15 years and is administered to approximately 4000 patients worldwide. Here we review the crystal structure of Cerezyme® and other recombinant forms of GlcCerase, as well as of their complexes with covalent and non-covalent inhibitors. We also discuss the stability of Cerezyme®, which can be altered by modification of its N-glycan chains with possible implications for improved ERT in Gaucher disease.

Keywords: Gaucher disease; glucosylceramide; lysosome; X-ray structure

About the article

Corresponding author

Received: 2008-05-08

Accepted: 2008-08-04

Published Online: 2008-09-11

Published in Print: 2008-11-01

Citation Information: Biological Chemistry, ISSN (Online) 1437-4315, ISSN (Print) 1431-6730, DOI: https://doi.org/10.1515/BC.2008.163.

Export Citation

Citing Articles

Here you can find all Crossref-listed publications in which this article is cited. If you would like to receive automatic email messages as soon as this article is cited in other publications, simply activate the “Citation Alert” on the top of this page.

Laura Smith, Stephen Mullin, and Anthony H.V. Schapira
Experimental Neurology, 2017
Marc N. Offman, Marcin Krol, Israel Silman, Joel L. Sussman, and Anthony H. Futerman
Journal of Biological Chemistry, 2010, Volume 285, Number 53, Page 42105
Thai Leong Yap, James M. Gruschus, Arash Velayati, Wendy Westbroek, Ehud Goldin, Nima Moaven, Ellen Sidransky, and Jennifer C. Lee
Journal of Biological Chemistry, 2011, Volume 286, Number 32, Page 28080
Nevein Philip Botross, Amgad Abadir Riad, Shanthi Viswanathan, Rusli Bin Nordin, and HN Lock
Scottish Medical Journal, 2014, Volume 59, Number 2, Page e1
You-Hai Xu, Ying Sun, Sonya Barnes, Gregory A. Grabowski, and Raphael Schiffmann
PLoS ONE, 2010, Volume 5, Number 5, Page e10750
Laercio Pol-Fachin, Marina Siebert, Hugo Verli, and Maria Luiza Saraiva-Pereira
Glycoconjugate Journal, 2016, Volume 33, Number 2, Page 237
Saki Sultana, Nhu Y. Truong, Douglas B. Vieira, Jasper G.D. Wigger, A. Michael Forrester, Chansey J. Veinotte, Jason N. Berman, and Aarnoud C. van der Spoel
Zebrafish, 2016, Volume 13, Number 3, Page 177
Hongping Ye, John Hill, Ashley C. Gucinski, Michael T. Boyne, and Lucinda F. Buhse
The AAPS Journal, 2015, Volume 17, Number 2, Page 405
Lisa Kain, Bill Webb, Brian L. Anderson, Shenglou Deng, Marie Holt, Anne Costanzo, Meng Zhao, Kevin Self, Anais Teyton, Chris Everett, Mitchell Kronenberg, Dirk M. Zajonc, Albert Bendelac, Paul B. Savage, and Luc Teyton
Immunity, 2014, Volume 41, Number 4, Page 543
Juliana Branco Novo, Ligia Morganti, Ana Maria Moro, Adriana Franco Paes Leme, Solange Maria de Toledo Serrano, Isaias Raw, and Paulo Lee Ho
Journal of Biomedicine and Biotechnology, 2012, Volume 2012, Page 1
Boris Brumshtein, Matilde Aguilar-Moncayo, Juan M. Benito, José M. García Fernandez, Israel Silman, Yoseph Shaaltiel, David Aviezer, Joel L. Sussman, Anthony H. Futerman, and Carmen Ortiz Mellet
Organic & Biomolecular Chemistry, 2011, Volume 9, Number 11, Page 4160
Erich Gulbins
Biological Chemistry, 2008, Volume 389, Number 11
Cedric E. Bobst, John J. Thomas, Paul A. Salinas, Philip Savickas, and Igor A. Kaltashov
Protein Science, 2010, Volume 19, Number 12, Page 2366
Martin D Witte, Wouter W Kallemeijn, Jan Aten, Kah-Yee Li, Anneke Strijland, Wilma E Donker-Koopman, Adrianus M C H van den Nieuwendijk, Boris Bleijlevens, Gertjan Kramer, Bogdan I Florea, Berend Hooibrink, Carla E M Hollak, Roelof Ottenhoff, Rolf G Boot, Gijsbert A van der Marel, Herman S Overkleeft, and Johannes M F G Aerts
Nature Chemical Biology, 2010, Volume 6, Number 12, Page 907
C. P. Phenix, B. P. Rempel, K. Colobong, D. J. Doudet, M. J. Adam, L. A. Clarke, and S. G. Withers
Proceedings of the National Academy of Sciences, 2010, Volume 107, Number 24, Page 10842
Sarah M. Lo, Joseph McNamara, Margherita R. Seashore, and Pramod K. Mistry
Journal of Inherited Metabolic Disease, 2010, Volume 33, Number S3, Page 429
Juliana Branco Novo, Maria Leonor Sarno Oliveira, Geraldo Santana Magalhães, Ligia Morganti, Isaías Raw, and Paulo Lee Ho
Molecular Biotechnology, 2010, Volume 46, Number 3, Page 279
B. Brumshtein, P. Salinas, B. Peterson, V. Chan, I. Silman, J. L Sussman, P. J Savickas, G. S Robinson, and A. H Futerman
Glycobiology, 2010, Volume 20, Number 1, Page 24
Melissa R. Landon, Raquel L. Lieberman, Quyen Q. Hoang, Shulin Ju, Jose M. M. Caaveiro, Susan D. Orwig, Dima Kozakov, Ryan Brenke, Gwo-Yu Chuang, Dmitry Beglov, Sandor Vajda, Gregory A. Petsko, and Dagmar Ringe
Journal of Computer-Aided Molecular Design, 2009, Volume 23, Number 8, Page 491
Boris Brumshtein, Matilde Aguilar-Moncayo, M. Isabel García-Moreno, Carmen Ortiz Mellet, José M. García Fernández, Israel Silman, Yoseph Shaaltiel, David Aviezer, Joel L. Sussman, and Anthony H. Futerman
ChemBioChem, 2009, Volume 10, Number 9, Page 1480

Comments (0)

Please log in or register to comment.
Log in