Trends in Glycoscience and Glycotechnology
Online ISSN : 1883-2113
Print ISSN : 0915-7352
ISSN-L : 0915-7352
Volume 30, Issue 173
Displaying 1-7 of 7 articles from this issue
  • Tsuyoshi Ichiyanagi
    2018 Volume 30 Issue 173 Pages E25-E32
    Published: March 25, 2018
    Released on J-STAGE: March 25, 2018

    Lipopolysaccharides (LPSs) and lipooligosaccharides (LOSs) are target molecules for the development of vaccines against pathogenic Gram-negative bacterial infections. The chemical synthesis of the core oligosaccharides of LPSs/LOSs remains a major challenge. Herein, recent reports of the chemical synthesis of inner-core oligosaccharides containing 3-deoxy-D-manno-oct-2-ulosonic acid are reviewed.

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  • Kazuhiro Aoki, Michael Tiemeyer
    2018 Volume 30 Issue 173 Pages E33-E40
    Published: March 25, 2018
    Released on J-STAGE: March 25, 2018

    The ST3GAL5 enzyme adds sialic acid in α-3 linkage to lactosylceramide, producing ganglioside GM3, a glycosphingolipid (GSL) enriched in neural tissue. Four human mutations in the ST3Gal5 gene have been identified that each result in a complete lack of ganglioside GM3 and collateral changes in the biosynthesis of other glycosphingolipids. Patients carrying any of these mutations are born normally but soon suffer neurodevelopmental delays. The most severe of the mutant ST3GAL5 alleles cause intractable seizures, sensory disruptions, altered skin pigmentation, microcephaly, and distinct neurological sequelae. Unlike diseases in glycosphingolipid degradation pathways (lysosomal storage disorders), very little is known regarding the molecular and pathophysiologic consequences of altered GSL biosynthesis. Therefore, these human biosynthetic deficiencies offer an opportunity to assess the broad glycomic impact of altered GSL profiles. In this review, we describe the glycomic consequences of one human ST3GAL5 mutant disorder, known as Salt and Pepper Syndrome, and discuss the value of stem cell and whole organism models for investigating disease mechanisms. We highlight a comprehensive glycomic approach that continues to provide data challenging our understanding of the regulatory mechanisms that control expression of the entire glycome.

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