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Mol Biol Cell. 2010 Nov 15;21(22):3773-5. doi: 10.1091/mbc.E10-05-0417.

A fascination with sugars.

Author information

  • Washington University in St. Louis, Internal Medicine, Saint Louis, MO 63110, USA. skornfel@im.wustl.edu

Abstract

We now recognize that a large number of membrane and soluble proteins contain covalently linked oligosaccharides that exhibit a vast array of structures and participate in a wide variety of biological processes. Nowhere is this better illustrated than the mannose 6-phosphate (Man-6-P) recognition system that mediates the trafficking of newly synthesized acid hydrolases to lysosomes in higher eukaryotes. The Asn-linked high-mannose oligosaccharides of these hydrolases facilitate folding of the nascent proteins in the endoplasmic reticulum via interaction with lectin-type chaperones and after phosphorylation in the Golgi, function as ligands for binding to Man-6-P receptors, a critical step in their transport to lysosomes. Failure to synthesize the Man-6-P recognition marker results in a serious lysosomal storage disease, one of a growing number of genetic conditions, termed congenital disorders of glycosylation, that result from faulty glycan biosynthesis.

PMID:
21079006
[PubMed - indexed for MEDLINE]
PMCID:
PMC2982096
Free PMC Article

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