Genetic Disorders of Glycan Degradation

Hudson H. Freeze; Richard Steet; Tadashi Suzuki; Taroh Kinoshita; Ronald L. Schnaar

doi:10.1101/glycobiology.4e.44

Genetic Disorders of Glycan Degradation

Review

In: Essentials of Glycobiology [Internet]. 4th edition. Cold Spring Harbor (NY): Cold Spring Harbor Laboratory Press; 2022. Chapter 44.

Authors

Hudson H. Freeze, Richard Steet, Tadashi Suzuki, Taroh Kinoshita, Ronald L. Schnaar

Book Editors

Book Affiliations

¹ Distinguished Professor of Medicine and of Cellular & Molecular Medicine, Co-Director, Glycobiology Research and Training Center, University of California, San Diego, La Jolla, California, USA
² Professor of Surgery, Beth Israel Deaconess Medical Center, Director, National Center for Functional Glycomics, Harvard Medical School, Boston, 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, Professor of Cell Biology, Albert Einstein College of Medicine, Bronx, New York, USA
⁵ Sr. Eminent Scholar in Drug Discovery, Georgia Research Alliance William Henry Terry Professor of Biochemistry & Molecular Biology, Complex Carbohydrate Research Center, University of Georgia, Athens, Georgia, USA
⁶ Professor of Mycology, ETH Zürich, Institute of Microbiology, Department of Biology, Zürich, Switzerland
⁷ Distinguished Research Professor, Georgia Athletics Association Professor in Complex Carbohydrate Research, Professor of Biochemistry and Molecular Biology, Complex Carbohydrate Research Center, University of Georgia, Athens, Georgia, USA
⁸ Endowed Chair and Professor, Yabumoto Department of Intractable Disease Research, Research Institute for Microbial Diseases, Immunology Frontier Research Center, Osaka University, Osaka, Japan
⁹ Distinguished Professor of Molecular Sciences, Macquarie University, Sydney, Australia and Principal Research Leader, Institute for Glycomics, Griffith University, Gold Coast, Australia
¹⁰ Professor and Eminent Scholar Emeritus, Complex Carbohydrate Research Center, University of Georgia, Athens, Georgia, USA
¹¹ Professor, Departments of Pharmacology and Neuroscience, Johns Hopkins University, Baltimore, Maryland, USA
¹² Director of Max-Planck-Institute of Colloids and Interfaces, Potsdam, Germany, Professor, Freie Universität, Berlin, Germany

PMID: 35536982
Bookshelf ID: NBK579991
DOI: 10.1101/glycobiology.4e.44

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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