Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2012 Dec 19:2012:506160.
doi: 10.5402/2012/506160. eCollection 2012.

Ganglioside biochemistry

Thomas Kolter  1
Affiliations
Review

Ganglioside biochemistry

Thomas Kolter. ISRN Biochem. .

Abstract

Gangliosides are sialic acid-containing glycosphingolipids. They occur especially on the cellular surfaces of neuronal cells, where they form a complex pattern, but are also found in many other cell types. The paper provides a general overview on their structures, occurrence, and metabolism. Key functional, biochemical, and pathobiochemical aspects are summarized.

PubMed Disclaimer

Figures

Figure 1
Figure 1
Sialic acids.
Figure 2
Figure 2
Structure of GQ1b, one of the most abundant gangliosides in adult human brain, which is involved in long term potentiation, synaptic plasticity, and improvement of cognitive function [23].
Figure 3
Figure 3
GD1aGalNAc and GM4.
Figure 4
Figure 4
Formation of SSEA-4 from globotriaosylceramide (Gb3Cer). SSEA, stage-specific embryonic antigen; T, transferase; FUT, fucosyltransferase (modified from [24]).
Figure 5
Figure 5
Examples for gangliosides from echinoderms: LG-2 from the starfish Astropecten latespinosus [47] and LLG-5 from the starfish Linckia laevigata [–50].
Figure 6
Figure 6
Representative structures of sphingoid bases, in this case (d18:1), (d18:0), and (t18:0).
Figure 7
Figure 7
Representative structures of gangliosides that are abundant in adult human brain.
Figure 8
Figure 8
Example of a ganglioside lactone.
Figure 9
Figure 9
Ceramide biosynthesis, shown for a ceramide with C18 sphingosine. R = alkyl; chain length depend on the availability of the corresponding acyl CoAs and the identity of the ceramide synthase.
Figure 10
Figure 10
Reaction of β1,3-N-acetyl-glucosaminyltransferase (β3GlcNAcT) and examples of downstream gangliosides of the neolacto series.
Figure 11
Figure 11
Structures of hematosides.
Figure 12
Figure 12
Ganglioside biosynthesis (modified from [141]).
Figure 13
Figure 13
Structure of a c-series ganglioside.
Figure 14
Figure 14
Bis(monoacylglycero)phosphate with two oleic acid moieties.
Figure 15
Figure 15
Lysosomal membrane pools.
Figure 16
Figure 16
Lysosomal ganglioside degradation pathway (modified from [233]). Names of inherited diseases (in boxes), enzymes, and lipid-transfer proteins required in vivo are given.
Figure 17
Figure 17
Synthetic substrates for the determination of β-hexosaminidase A (R = SO3 −) and β-hexosaminidases A + B (R = H).
Figure 18
Figure 18
LysoGM2 (n = 12, 14).
Figure 19
Figure 19
Structure of Liga20, a semitruncated and halogenated GM1 analog that can pass the blood-brain barrier.
Figure 20
Figure 20
Schematic ganglioside uptake by cultured cells (modified from [306]).
Figure 21
Figure 21
Example of a GM1 analog [307] spin labelled with a 4,4-dimethyl-oxazolidine-1-oxyl- (DOXYL-) residue and a fluorescent GM1 analog used for STED microscopy [308].
See this image and copyright information in PMC

References

    1. Fahy E., Subramaniam S., Brown H. A., et al. A comprehensive classification system for lipids. Journal of Lipid Research. 2005;46(5):839–861. doi: 10.1194/jlr.E400004-JLR200. - DOI - PubMed
    1. Wickramasinghe S., Medrano J. F. Primer on genes encoding enzymes in sialic acid metabolism in mammals. Biochimie. 2011;93:1641–1646. doi: 10.1016/j.biochi.2011.06.002. - DOI - PubMed
    1. Miyagi T., Yamaguchi K. Sialic acids. In: Kamerling J. P., Boons G. J., Lee Y. C., Suzuki A., Taniguchi N., Voragen A. G. J., editors. Comprehensive Glycoscience. Vol. 3. Oxford, UK: Elsevier; 2007. pp. 297–322.
    1. Klenk E. Über die Ganglioside, eine neue Gruppe von zuckerhaltigen Gehirnlipoiden. Hoppe-Seyler's Zeitschrift für Physiologische Chemie. 1942;273:76–86.
    1. Klenk E. Über die Natur der Phosphatide und anderer Lipide des Gehirns und der Leber bei der Niemann-Pick'schen Krankheit. Zeitschrift für Physiologische Chemie. 1935;235:24–25.

LinkOut - more resources