Genetic Disorders of Glycan Degradation

Hudson H. Freeze; Taroh Kinoshita; Ronald L. Schnaar

doi:10.1101/glycobiology.3e.044

Genetic Disorders of Glycan Degradation

Review

In: Essentials of Glycobiology [Internet]. 3rd edition. Cold Spring Harbor (NY): Cold Spring Harbor Laboratory Press; 2015–2017. Chapter 44.

2017.

Authors

Hudson H. Freeze, Taroh Kinoshita, Ronald L. Schnaar

Book Editors

Book Affiliations

¹ Distinguished Professor of Medicine and Cellular & Molecular Medicine, Co-Director, Glycobiology Research and Training Center, University of California, San Diego, La Jolla, California, USA
² Professor of Surgery, Director, National Center for Functional Glycomics, Harvard Medical School, Cambridge, Massachusetts, USA
³ Distinguished Professor of Cellular & Molecular Medicine, Co-Director, Glycobiology Research and Training Center, University of California, San Diego, La Jolla, California, USA
⁴ Horace W. Goldsmith Foundation Chair and Professor of Cell Biology, Albert Einstein College of Medicine, New York, New York, USA
⁵ Professor and Chairman of Biological Chemistry, Johns Hopkins University, Baltimore, Maryland, USA
⁶ Professor of Mycology, ETH Zürich, Zürich, Switzerland
⁷ Regents Professor and Director, Complex Carbohydrate Research Center, University of Georgia, Athens, Georgia, USA
⁸ Professor, Laboratory of Immunoglycobiology, WPI Immunology Frontier Research Center, Osaka University, Osaka, Japan
⁹ Professor of Glycoproteomics and Glycomics, Macquarie University and Institute for Glycomics, Griffith University, Sydney, Australia
¹⁰ Professor and Eminent Scholar, University of Georgia, Athens, Georgia, USA
¹¹ Professor, Departments of Pharmacology and Neuroscience, Johns Hopkins University, Baltimore, Maryland, USA
¹² Director, Max-Planck-Institute of Colloids and Interfaces, Potsdam, Germany

Excerpt

This chapter explores degradation and turnover of glycans in lysosomes, especially with respect to human genetic disorders, with representative glycans, illustrating features unique to different pathways. Degradation of oligomannosyl N-glycans removed from misfolded, newly synthesized glycoproteins is covered in Chapters 39 and 45.

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