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Acidic Glycosphingolipid

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Ganglioside ガングリオシド

Ganglioside is a glycosphingolipid with one or more sialic acid. It was named by Klenk E. in 1942 as the major sphingolipid in ganglion, a nerve cell cluster.<ref>Sweeley CC, Siddiqui B. "Chemistry of Mammalian Glycolipids" Chapter 6 in The Glycoconjugates Volume I: Mammalian Glycoproteins and Glycolipids (Ed. Horowitz MI, Pigman W) 1977 Elsevier Inc. ISBN 978-0-12-356101-5</ref> In vertebrate, close to 190 gangliosides have been identified.<ref>Yu RK, Tsai YT, Ariga T, Yanagisawa M. (2011) Structures, biosynthesis, and functions of gangliosides--an overview" J Oleo Sci. 60(10):537-44. PMID 21937853</ref> They reside on lipid rafts of cell membranes, and involve in many cellular functions. Major structures with sialic acids include ganglio, lacto, and neolacto series.

酸性糖であるシアル酸を含む糖脂質をガングリオシドと呼びます。神経細胞の集まりである神経核 (ganglion) に多いことから Klenk E.により1942年に命名されました。 脊椎動物では現在およそ190種のガングリオシドが見出されています。ガングリオシドは細胞膜の脂質ラフトに存在し、多くの生理機能に関わっています。シアル酸の結合する主要系列はガングリオ、ラクト、ネオラクト等です。


Svennerholm Notation スヴェンネルホルム表記

The notation was proposed by the Swedish scientist, Lars Svennerholm. Each alpha-numeric digit stands for the number of sialic acids or sugars.

スウェーデンの科学者 Lars Svennerholm が導入した記法です。各桁の数字やアルファベットはシアル酸の数や糖の数を意味します。

Abbreviation GM GD GT GQ GP
Number of Sialic Acid 1 2 3 4 5
Suffix Number 1 2 3 4
Core Gg4Cer Gg3Cer LacCer GalCer
Suffix Alphabet (only for Gg4Cer core) a b c
Number of Sialic Acid Attached to IIGal 1 2 3
GQ1b ... 4 sialic acids (GQ), 4 sugars (GQ1), 2 sialic acids attached to the 2nd galactose (GQ1b)
GM2 ... 1 sialic acids (GM), 3 sugars (GM2).

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Sulfatide スルファチド

More than ten compounds of sulfoglycolipids (glycosphingolipid sulfate, sulfatide, SM4) are identified by differences in sulfonated sugar chains. Among them 3-O-Sulfated galactosylceramide(GalCer) is called galactosylsulfatide, and is present in myelin sheath like GalCer. <ref>Ishizuka, I. “Chemistry and functional distribution of sulfoglycolipids” Prog. Lipid Res.1997; 36:245-319 PMID 9640458 </ref> The existence of glucosylsufatide, 3-O-sulfated glucosylceramide was reported in rat kidney.<ref>Iida N, Toida T, Kushi Y, Handa S, Fredman P, Svennerholm L, Ishizuka I. A sulfated glucosylceramide from rat kidney. J Biol Chem. 1989 5;264:5974-80.PMID 2925645 </ref>

Sulfatides are present in various organs, such as nervous system, kidney, respiratory organ and digestive tract. The physiological functions of sulfatides are related to myelinogenesis, diabetes, immune system, thrombosis, bacterial and virus infection. <ref> Takahashi T, Suzuki T. “Role of sulfatide in normal and pathological cells and tissues” J Lipid Res. 2012; 53: 1437–1450, PMID 22619219 Takahashi T, Suzuki T. “Role of sulfatide in influenza A virus replication” Biol Pharm Bull. 2015; 38:809-16.PMID 26027821 </ref>

Lysosomal storage disease, metachromatic leukodystrophy, is a congenital metabolic disorder, and is caused by deficiency of lysosomal enzyme arylsulfatase A or its activator.<ref>Mirzaian M, Kramer G, Poorthuis BJ. “Quantification of sulfatides and lysosulfatides in tissues and body fluids by liquid chromatography-tandem mass spectrometry”J Lipid Res. 2015; 56:936-43. PMID 25632048 </ref>



リソソーム病である異染性白質ジストロフィー(metachromatic leukodystrophy)という先天性代謝異常症は、スルファチド分解酵素(ASA)あるいは活性化因子の欠損か低下により起こります。

Metabolic pathway<ref>Honke K. Biosynthesis and biological function of sulfoglycolipids. Proc Jpn Acad Ser B Phys Biol Sci. 2013 89:129-38. PMID 23574804</ref>

Cerebroside sulfotransferase (CST) + 3’-phosphoadenosine-5’-phosphosulfate(PAPS)
Galactosylceramide → galactosylsulfatide
Arylsulfatase A (ASA) + Saposin B (activator)
Galctoslsulfatide → Galactosylceramide


Acidic Modifiers 酸性基

Sialic acid シアル酸

Sialic acid is a collective noun and comprises a family of derivatives of neuraminic acid (5-amino- 3,5-dideoxy- D-glycero- D-galacto- nonulosonic acid)<ref>Schauer R "Sialic Acids - Chemistry, Metabolism and Function" Springer-Verlag, 1982.ISBN 978-3-7091-8680-0 </ref>. Nowadays, there are over 30 derivatives of neuraminic acid, including the followings.

  • N-acetyl neuraminic acid (NeuAc)
  • N-glycolyl neuraminic acid (NeuGc)
  • deamino neuraminic acid (Kdn: 3-deoxy-D-glycero-D-galacto-nonulosonic acid)

NeuGc is different from NecAc only by a single oxygen, and is produced by N-acetylneuraminic acid hydroxylase (CMAH). This gene is generally conserved among vertebrates but human, sauropsids (birds and reptiles), and monotreme lack this gene <ref>Irie A, Koyama S, Kozutsumi Y, et al. "The molecular basis for the absence of N-glycolylneuraminic acid in humans" J Biol Chem 273:15866–15871, 1998 PMID 9624188</ref><ref>Schauer R, Srinivasan GV, Coddeville B, et al. "Low incidence of N-glycolylneuraminic acid in birds and reptiles and its absence in the platypus" Carbohydr Res 344:1494–1500, 2009 PMID 19541293</ref> (therefore no NeuGc). Kdn is prominent in some fish but not in mammals.

シアル酸は、ノイラミン酸(5-アミノ-3,5-ジデオキシ-D-グリセロ-D-ガラクト-ノヌロン酸)に由来する物質の総称です。 現在、ノイラミン酸には以下を含む30 種以上の誘導体が知られています。

  • N-アセチルノイラミン酸 (NeuAc)
  • N-グリコリルノイラミン酸 (NeuGc)
  • デアミノノイラミン酸 (Kdn)


N-acetyl neuraminic acid (Neu5Ac) N-glycolyl neuraminic acid (Neu5Gc) deamino neuraminic acid (Kdn)

Derivatives include the followings for the above three types.<ref>Angata T, Varki A "Chemical diversity in the sialic acids and related alpha-keto acids: an evolutionary perspective" Chem Rev 102(2):439-469, 2002 PMID 11841250</ref> Such modifications are tissue- and organelle-specific.<ref>Butor C, Diaz S, Varki A "High level O-acetylation of sialic acids on N-linked oligosaccharides of rat liver membranes. Differential subcellular distribution of 7- and 9-O-acetyl groups and of enzymes involved in their regulation" J Biol Chem 268(14):10197-10206, 1993 PMID 8486687</ref>

  • 8-O-methylation, 9-O-methylation
  • 4-O-sulfation, 8-O-sulfation
  • O-acetylation on the hydroxyl groups at C4, C7, and C9

誘導体には、上記の 3 構造全てにおいて以下が挙げられます。修飾は臓器や細胞小器官特異的におこなわれます。

  • 8-O-メチル化、9-O-メチル化
  • 4-O-硫酸化、8-O-硫酸化
  • C4, C7 または C9 における O-アセチル化


  • Gunnar Blix found a novel acidic aminosugars from salivary gland mucin in 1936 and later named it sialic acid (1938)<ref> Lundblad A "Gunnar Blix and his discovery of sialic acids. Fascinating molecules in glycobiology" Ups J Med Sci. 2015; 120:104–112 PMID 25921326</ref>. (Dried salivary mucin contains 2% sialic acid.)
  • Ernst Klenk identified "Substanz X" from the brain (neuro) of Tay-Sach's disease patient (1935) and later identified a polyhydroxyamino carboxylic acid, and called it neuraminic acid (1941).
  • Both substances are found identical, and the correct structure was postulated by Alfred Gottshalk in 1955 <ref>Gottschalk A. "Structural Relationship between Sialic Acid, Neuraminic Acid and 2-Carboxy-Pyrrole" Nature 1955; 176, 881 - 882 doi:10.1038/176881a0 </ref>
  • In the same period, Tamio Yamakawa and Sakaru Suzuki identified a novel ganglioside from horse erythrocyte stroma and named it hematoside (1951), now called GM3<ref>Kanfer JN and Hakomori S "Sphingolipid biochemistry (Handbook of Lipid Research 3)" Chapter 1, Springer Science & Business Media, 2012 ISBN 978-1475703986</ref>.

  • Gunnar Blixが1936年に唾液(sialo)のムチンから新規の酸性アミノ糖を見つけ、後にこれをシアル酸と名づけました(1938)。
  • Ernst Klenkはテイサックス病患者の脳から「物質X」を見つけ (1935)、後に加水分解によって水酸化アミノカルボン酸であるノイラミン酸を抽出しました(1941)。
  • 両者は同一物質であることがわかり、Alfred Gottshalkが1955年に正しい構造を提出しました。
  • Klenkとほぼ同時代に山川民夫と鈴木旺は馬の赤血球からヘキソースアミンを含まないガングリオシドを抽出し、1951年ヘマトシドと名付けました(現在のGM3)。



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