Abstract
In the rough endoplasmic reticulum, first modifications on asparagine-linked oligosaccharides occur and include the removal of all three glucose residues by α-glucosidases I (GIsI) and II (GIsII) and some mannose residues by ER α-mannosidases I (ER Man I) and II (ER Man II), as illustrated in the scheme. These trimming reactions and the resulting specific oligosaccharides are important for protein quality control. Proper folding and correct assembly of glycoproteins are constantly monitored by a quality control machinery composed of chaperones, lectins such as calnexin and calreticulin, glucosidase II and UDP-glucose: glycoprotein glucosyltransferase (for additional details, cf. Fig. 24). On the other hand, defective oligosaccharide trimming has been recognized as the cause of congenital disorders of glycosylation.
This is a preview of subscription content, log in via an institution to check access.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Ellgaard L and Helenius A (2003) Quality control in the endoplasmic reticulum. Nat Rev Mol Cell Biol 4: 181
Freeze HH (2006) Genetic defects in the human glycome. Nat Rev Genet 7: 537
Jakob CA, Burda P, Roth J, and Aebi M (1998) Degradation of misfolded endoplasmic reticulum glycoproteins in Saccharomyces cerevisiae is determined by a specific oligosaccharide structure. J Cell Biol 142: 1223
Hennet T (2009) Diseases of glycosylation. In: The Sugar Code. Fundamentals of Glycosciences (Gabius H-J, ed). Weinheim: Wiley/VCH, pp 365
Parodi AJ (2000) Protein glucosylation and its role in protein folding. Annu Rev Biochem 69: 69
Roth J, Yam GH, Fan J, Hirano K, Gaplovska-Kysela K, Le Fourn V, Guhl B, Santimaria R, Torossi T, Ziak M, and Zuber C (2008) Protein quality control: the who’s who, the where’s and therapeutic escapes. Histochem Cell Biol 129: 163
Vembar SS and Brodsky JL (2008) One step at a time: endoplasmic reticulum-associated degradation. Nat Rev Mol Cell Biol 9: 944
Author information
Authors and Affiliations
Rights and permissions
Copyright information
© 2010 Springer-Verlag/Wien
About this chapter
Cite this chapter
Pavelka, M., Roth, J. (2010). Oligosaccharide Trimming, Reglucosylation, and Protein Quality Control in the Rough Endoplasmic Reticulum. In: Functional Ultrastructure. Springer, Vienna. https://doi.org/10.1007/978-3-211-99390-3_20
Download citation
DOI: https://doi.org/10.1007/978-3-211-99390-3_20
Publisher Name: Springer, Vienna
Print ISBN: 978-3-211-99389-7
Online ISBN: 978-3-211-99390-3
eBook Packages: Biomedical and Life SciencesBiomedical and Life Sciences (R0)