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==Cerebrosides==
<font size="+2">Cerebroside</font>
{{LB/Header}}
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{|
|{{LBS/Cer|O-<b>Glucose</b>}}  <b>GlcCer</b>
|{{LBS/Cer|O-<b>Galactose</b>}}  <b>GalCer</b>
|-
|colspan=2|
{{Twocolumn|
{{Twocolumn|
Monoglycosylceramides (or cerebrosides) are glycosphingolipids where a single sugar is attached to the ceramide structure. Especially, glucose-attached Glucosylceramide (GlcCer) and galactose-attached Galactosylceramide (GalCer) are widely observed in all kingdoms, and therefore we do not register them in LipidBank. Instead, we summarize their distribution with bibliographic references for biological species<ref>Itonori S, Sugita M. Glycophylogenetic Aspects of Lower Animals. in Comprehensive Glycoscience Vol.3, Biochemistry of glycoconjugate glycans. (J.P. Kamerling, ed.) Elsevier, Oxford (UK). pp. 253-84 (2007)</ref><ref>Yamakawa T, Nishimura S. (1966) Cerebroside composition in animal brain. Jpn J Exp 36:101-102.</ref>.
Monoglycosylceramides (or cerebrosides) are glycosphingolipids where a single sugar is attached to the ceramide structure. Especially, glucopyranose-attached Glucosylceramide (GlcCer) and galactopyranose-attached Galactosylceramide (GalCer) are widely observed in all kingdoms.
GlcCer and GalCer are not registered in LipidBank. Instead, we summarize their distribution with bibliographic references with species information.


<big>GlcCer</big> is widely observed not only in Animal, Plant or Fungi kingdoms, but also in Protozoa Kingdom. Ceramides are formed from fatty acids and long-chain bases, and their structures (hydrocarbon chain length, the number and position of double bonds, existence of hydroxy and other modification groups) may vary, and become complex especially in Plant and Fungi Kingdoms.
In Animal Kingdom, GlcCer is the basic structure for different derivative series such as Globo, Lacto, and Ganglio Series. In Protostomia, GlcCer is the major ceramide in its nerve tissue, e.g. shrimp brain <ref>Okamura N, Stoskopf M, Hendricks F, Kishimoto Y. (1985) Phylogenetic dichotomy of nerve glycosphingolipids. Proc Natl Acad Sci, 82:6779-6782</ref>, and the amide-linked fatty acids do not contain hydroxy groups. In Mammalia (of Deuterostomia), GlcCer is major in the spleen, blood, and skin. Gaucher's disease is an inborn metabolic disorder in which GlcCer accumulates in the liver and spleen because of the deficiency of the enzyme glucocerebrosidase to dissociate glucose from the ceramide structure.
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モノグリコシルセラミド (またはセレブロシド) はセラミドに糖が1個エステル結合したスフィンゴ糖脂質である。特にグルコースが結合したグルコシルセラミド (GlcCer) とガラクトースが結合したガラクトシルセラミド (GalCer) は生物界に広く分布するのでデータベース中には掲載せず、ここに解説文と生物種ごとの報告例を掲載する。
モノグリコシルセラミド (またはセレブロシド) はセラミドに糖が1個エステル結合したスフィンゴ糖脂質です。特にグルコース（ピラノース型）が&beta;結合したグルコシルセラミド (GlcCer) とガラクトース（ピラノース型）が&beta;結合したガラクトシルセラミド (GalCer) は生物界に広く分布します。データベース中には掲載せず、ここに解説と生物種ごとの報告例を掲載します。
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==Glucosylceramide (GlcCer)==
 
{{Twocolumn|
GlcCer is widely observed not only in Animal, Plant or Fungi kingdoms, but also in Protozoa Kingdom<ref>Tan RX, Chen JH. "The cerebrosides" Nat Prod Rep. 2003;20:509-34.PMID 14620845,  Yu R.K. Yanagisawa M. Ariga T. Glycosphingolipid Structures,in Comprehensive Glycoscience Vol.1,pp.  73-122,doi 10.1016/B978-044451967-2/00003-9,  Itonori S, Sugita M. Glycophylogenetic Aspects of Lower Animals. in Comprehensive Glycoscience Vol.3,  pp. 253-84, doi 10.1016/B978-044451967-2/00050-7 (J.P. Kamerling, ed.) Elsevier, Oxford (UK). (2007)</ref>. Ceramide structures (hydrocarbon chain length, the number and position of double bonds, existence of hydroxy and other modification groups) may vary, and become complex especially in Plant and Fungi Kingdoms, and Protostomia <ref>Sphingoid base chain may terminate with an isopropyl group in spongia, annelida, echinodermata, and bacteria. Terminal isobutyl or sec-pentyl group has been found only in echinodermata. Methylated or cyclopropane-containing sphingoid base is found in spongia, echinodermata, protochordata, and fungi.</ref>.
In Animal Kingdom, GlcCer is the basic structure for different derivative series such as Globo, Lacto, and Ganglio Series. In Protostomia, GlcCer is the major ceramide in its nerve tissue, e.g. shrimp brain <ref>Okamura N, Stoskopf M, Hendricks F, Kishimoto Y.  "Phylogenetic dichotomy of nerve glycosphingolipids" Proc Natl Acad Sci, 1985 82:6779-6782 PMID 3863128 </ref>, and its amide-linked fatty acids do not contain hydroxy groups.  GlcCer in plants contains many &alpha;-hydroxy fatty acids.  In some cold-resistant plants &omega;9-unsaturated fatty acids are detected. In Mammalia (of Deuterostomia), GlcCer is major in the spleen, blood, and skin.
 
Gaucher's disease is an inborn metabolic disorder in which GlcCer accumulates in the liver and spleen because of the deficiency of the enzyme glucocerebrosidase to dissociate glucose from the ceramide structure <ref> Brady RO, Kanfer JN, Shapiro D. "Metabolism of glucocerebrosides. II. Evidence of an enzymatic deficiency in Gaucher's desease" Biochem Biophys Res Commun. 1965;18:221-5 PMID 14282020 </ref>.
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'''GlcCer'''は系統発生的にみると動物の各組織に広く分布するだけでなく、植物界や菌界、原生生物界にも含まれます。セラミド構造（炭化水素の鎖長、二重結合の数と位置、水酸基の有無、修飾基の有無など）の組み合わせは多岐にわたり、特に植物界や菌界、旧口動物のセラミドは複雑です。動物界において、GlcCerはさまざまな糖鎖を持つ糖脂質の基本構造になり、グロボ系列、ラクト系列、ガングリオ系列などが生成されます。旧口（前口）動物の神経系にはGlcCerが多く含まれ（例えばエビ）、セラミド骨格の脂肪酸に水酸基を含みません。対して、植物は &alpha;-水酸化の脂肪酸が多く、耐冷性植物の一部からは &omega;9-不飽和の脂肪酸も見いだされます。後口動物の哺乳綱において、GlcCerは、脾臓、血液、皮膚などに多く含まれます。
 
先天性代謝異常症であるゴーシェ (Gaucher) 病は、GlcCerを分解するグルコセレブロシダーゼが欠損し、肝臓や脾臓にGlcCerが蓄積して発症します。
}}
 
==Galactosylceramide (GalCer)==
{{Twocolumn|
GalCer has been observed in Eubacterial Domain, but not in Protozoa, Plant, or Fungi (except Ascomycota). It is widely observed in Animal Kingdom, but not in Ecdysozoa (cuticle-shedding animals) of Protostomia. Its ceramide structure differs widely as in GlcCer but not so complex as GlcCer in Plant and Fungi. GalCer is the basic structure for sulfatide (3-O-sulfogalactosylceramide or SM4) and Gala Series often found in Annelida (segmented worm). In Mammalia (of Deuterostomia), GalCer is major in the nerve tissue, especially myelin sheath, whose amido-linked fatty acids are hydroxylated at the C-2 position for more than 50%.
 
Krabbe disease (globoid cell leukodystrophy) is an inborn metabolic disorder in which a small amount of psychosine (galactosylsphingosine, i.e., the lyso form of GalCer) induces demyelination because of the deficiency of the enzyme galactosylceramidase <ref>Suzuki K. "Globoid cell leukodystrophy (Krabbe's disease): update" J Child Neurol. 2003;18:595-603.PMID 14572137,  Suzuki K. "Evolving concept of the pathogenesis of globoid cell leukodystrophy (Krabbe disease)" Proc Jpn Acad Ser B Phys Biol Sci 2003; 79:1-8</ref>. &alpha;-GalCer, found from Porifera (marine sponge), in which galactose is &alpha;-bonded to ceramide (not the usual &beta;-bond), uniquely binds to human or mouse NKT cells and is considered a potent anti-tumor agent.<ref>Kitamura H, Iwakabe K, Yahata T, Nishimura S, Ohta A, Ohmi Y, Sato M, Takeda K, Okumura K, Van Kaer L, Kawano T, Taniguchi M, Nishimura T. "The natural killer T (NKT) cell ligand alpha-galactosylceramide demonstrates its immunopotentiating effect by inducing interleukin (IL)-12 production by dendritic cells and IL-12 receptor expression on NKT cells" J Exp Med. 1999; 5;189(7):1121-8. PMID 10190903 </ref> Recently, &alpha;-linked GlcCer and GalCer were reported in NKT cells in trace amount<ref>Kain L et al. "The Identification of the Endogenous Ligands of Natural Killer T Cells Reveals the Presence of Mammalian α-Linked Glycosylceramides" Immunity 41(4), 543-554, 2014 PMID 25367571</ref>.
 
'''GalCer'''は真正細菌に存在しますが、原生生物界、植物界、菌界（子嚢菌を除く）では報告がありません。動物界に広く分布しますが旧口動物である脱皮動物には確認されていません。セラミド構造は、GlcCerと同様に多様ですが、植物や菌類のGlcCerほど複雑ではありません。GalCerはスルファチド (3-O-硫酸化ガラクトシルセラミドまたはSM4) や環形動物門に多く含まれるガラ系列の基本構造です。
後口動物である哺乳綱では神経系、特にミエリン鞘にGalCerが多く含まれ、その脂肪酸の50%以上は、C2位が水酸化されています。
 
クラッベ病（グロボイド細胞ロイコジストロフィー; GLD）はガラクトシルセラミダーゼの欠損により微量のサイコシン（ガラクトシルスフィンゴシン、つまり GalCer のリゾ体）が蓄積して脱髄（ミエリンの破壊）を起こす遺伝性疾患です。また、海綿動物門に発見された&alpha;-GalCerは、ガラクトースがセラミドに（通常の&beta;でなく）&alpha;結合しており、ヒトやマウスのナチュラルキラーT (NKT) 細胞に特異的に結合し抗腫瘍効果がみられます。&alpha;結合型の GlcCer および GalCer はNKT細胞に微量に存在します。
}}
 
<references/>
 
==Cerebrosides in Mammals==
 
====Lipids in Heart Muscle====
 
{| class="wikitable collapsible collapsed"
|-
!
! Lipid (% wet weight)
! Phospholipid (% dry weight)
! Phosphatidylethanolamine<br/>PE (% dry weight)
! Phosphatidylcholine<br/>PC (% dry weight)
! Sphingomyelin (% dry weight)
! '''Cerebroside''' (% dry weight)
! Total cholesterol (% dry weight)
|-
! Human
| 8.3
| 6.3 - 7.5
| 1.4 - 2.8
| 3.3 - 5.7
| 0.2 - 0.5
| -
| -
|-
! Cow
| 3.1 - 19.5
| 7.4 - 11.8
| 5.3
| 4
| 0.5
| 2
| 0.4 (free)
|-
! Rat
| 0.4 - 2.4
| 5 - 8
| 0.84 (% wet)
| 2 (% wet)
| 0.14 (% wet)
| 1.4
| 0.4 - 0.6
|-
|colspan="8"|生化学データブック[I] 14. 臓器・組織の成分、心臓　脂質 p1636 1981 日本生化学会編　東京化学同人　ISBN 978-4-807901-74-6 
|}
 
====Lipids in Rat Testis====
{| class="wikitable collapsible collapsed"
|-
! Fraction
! Total Lipid (mg/mg protein)
! Phosphate (ug/mg protein)
! Phospholipid<br/>(% total lipid)
! Neutral Lipid<br/>(% total lipid)
! '''Cerebroside''' (% total lipid)
! Sulfated glycolipid<br/>(nmol/g total weight)
|-
! Total testis
| 0.40
| 62.88
| 62.9
| 34.6
| 2.5
| 556 &plusmn; 34
|-
! Golgi
| 1.26
| 18.49
| 58.3
| 39.4
| 2.3
| -
|-
! Remnant granule
| 0.60
| 19.67
| 29.5
| 68.0
| 2.5
| -
|-
! Microsome
| 0.63
| 53.21
| 83.8
| 14.5
| 1.7
| -
|-
! Lipid corpuscle
| > 19
| 12
| 1.5
| 98.5
| -
| -
|-
|colspan="7"|  Keenan TW et al. "Lipid composition of subcellular fractions from rat testis"Biochim Biophys Acta 1972; 270:433-443, PMID 4340988 Suzuki A et al. "Decrease of seminolipid content in the testes of rats with vitamin A deficiency determined by high performance liquid chromatography"J Biochem 1977; 82:461-7 PMID 91479 
|}
 
====Cerebroside and Sulfatide Ratio in Animal Brain ====
 
{| class="wikitable sortable collapsible collapsed"
|-
! Animal
! Kerasin<br/>(GalCer with straight fatty-acyl chain)
! Phrenosin<br/>(GalCer with hydroxy fatty-acyl chain)
! Sulfatide
|-
! Human cinerea (gray matter)
| 25
| 52
| 23
|-
! Human alba (white matter)
| 28
| 47
| 25
|-
! Cow cinerea
| 15
| 56
| 29
|-
! Cow alba
| 35
| 48
| 17
|-
! Dog
| 21
| 57
| 22
|-
! Cat
| 21
| 60
| 19
|-
! Mouse (4 weeks)
| 24
| 56
| 20
|-
! Rat (10 days)
| 35
| 42
| 23
|-
! Rat (35 days)
| 21
| 61
| 18
|-
! Rabbit
| 13
| 66
| 21
|-
! Guinea pig
| 28
| 54
| 18
|-
! Sheep
| 27
| 47
| 26
|-
! Chicken
| 44
| 37
| 19
|-
! Tuna
| 50
| 25
| 25
|-
! Shark
| 44
| 24
| 32
|-
| colspan="4" |  (weight %) From: Yamakawa T, Nishimura S. "Cerebroside composition in animal brain" Jpn J Exp 1966; 36:101-2 PMID 5297247 
|}
 
====Fatty acid composition of bovine brains====
 
{| class="wikitable sortable collapsible collapsed"
|-
!colspan="5"| Nonhydroxy fatty acid (adjusted GC peak %)
!colspan="3"| Hydroxy fatty acid
|-
! fatty acid || Ceramide || Sphingomyelin || Cerebroside || Sulfatide ||  fatty acid || Cerebroside || Sulfatide
|-
|12:0
|colspan="4"| - || 12h:0 || 0.4 || 0.3
|-
|13:0
|colspan="4"| - || 13h:0 || 0.2 || 0.2
|-
| 14:0 || 0.6 || 0.3 || 0.2 || 0.8 || 14h:0 || 0.8 || 3.9
|-
| 15:0 || 0.2 || 0.1 || tr. || 0.3 || 15h:0 || tr. || 0.3
|-
| 16:0 || 4.5 || 5.9 || 3.5 || 4.1 || 16h:0 || 0.1 || 0.7
|-
| 16:1 || 0.4 || tr. || 0.1 || 0.7 || 16h:1 || tr. || 1.7
|-
| 17:0 || tr. || 0.3 || tr. || tr. || 17h:0 || 0.2 || 0.9
|-
| 18:0 || 35.2 || 40.1 || 5.8 || 3.3 || 18h:0 || 16.2 || 12.3
|-
| 18:1 || 2.1 || 0.7 || 2.7 || 5.2 || 18h:1 || - || -
|-
| 19:0 || 0.1 || 0.1 || 0.2 || 0.3 || 19h:0 || 0.4 || 0.3
|-
| 20:0 || 1.0 || 0.4 || 0.6 || 0.3 || 20h:0 || 0.5 || 0.7
|-
| 20:1 || 0.5 || tr. || 0.3 || 0.4 || 20h:1 || - || -
|-
| 21:0 || tr. || tr. || - || - || 21h:1 || - || -
|-
| 22:0 || 3.3 || 3.5 || 3.6 || 3.5 || 22h:0 || 5.8 || 7.7
|-
| 22:1 || 0.5 || 0.6 || 0.6 || 0.6 || 22h:1 || - || -
|-
| 23:0 || 3.7 || 2.8 || 5.2 || 3.1 || 23h:0 || 8.9 || 6.5
|-
| 23:1 || 1.0 || 1.0 || 1.2 || 0.8 || 23h:1 || - || -
|-
| 24:0 || 9.8 || 10.7 || 17.4 || 17.8 || 24h:0 || 30.1 || 26.0
|-
| 24:1 || 18.4 || 24.6 || 39.3 || 39.5 || 24h:1 || 14.6 || 16.7
|-
| 25:0 || 1.4 || 1.5 || 3.6 || 2.2 || 25h:0 || 7.0 || 4.5
|-
| 25:1 || 3.0 || 3.3 || 6.2 || 5.2 || 25h:1 || 3.0 || 2.8
|-
| 26:0 || 2.3 || 0.6 || 1.7 || 2.3 || 26h:0 || 3.9 || 3.8
|-
| 26:1 || 3.6 || 2.6 || 7.6 || 8.5 || 26h:1 || 6.3 || 6.5
|-
| 27:0 || 2.9 || - || - || - || 27h:0 || - || -
|-
| 27:1 || 1.8 || - || tr. || - || 27h:1 || - || -
|-
|colspan="8"| From: O'Brien JS, Rouser G "The fatty acid composition of brain sphingolipids: sphingomyelin, ceramide, cerebroside, and cerebroside sulfate"J Lipid Res1964; 5:339-342.PMID 5873370 Per cent of FA of each column. No PUFA, trace amount of hydroxy FA in ceramide, no hydroxy FA in sphingomyelin. The total ratio of nonhydroxy to hydroxy acids in cerebroside and sulfatide were 42:58 and 77:23, respectively (77:13 in the original paper). Human brain has a similar composition.
|}
 
==Sphingolipids in All Kingdoms==
 
{{:Category:ScientificClassification/LBSC}}
 
==Biosynthesis==


'''GlcCer'''は系統発生的にみると動物の各組織に広く分布するだけでなく、植物界や菌界、原生生物界にも含まれる。セラミドは脂肪酸と長鎖塩基より構成される。脂肪酸と長鎖塩基の構造（炭化水素の鎖長、二重結合の数と位置、水酸基の有無、修飾基の有無など）の組み合わせは多岐にわたり、特に植物界や菌界のセラミドは複雑である。動物界において、GlcCerはさまざまな糖鎖を持つ糖脂質の基本構造になり、グロボ系列、ラクト系列、ガングリオ系列などが生成される。旧口（前口）動物の神経系にはGlcCerが多く含まれ（例えばエビ）、その脂肪酸にヒドロキシ酸は含まない特徴がある。後口動物の哺乳綱においては、脾臓、血液、皮膚などに多く含まれる。先天性代謝異常症であるゴーシェ (Gaucher) 病ではGlcCerを分解するグルコセレブロシダーゼが欠損しており、肝臓や脾臓にGlcCerが蓄積する。
{{Twocolumn|
In Mammalia, GlcCer is synthesized by ceramide glucosyltransferase (CGlcT) at the cytosolic membrane of the Golgi apparatus, and GalCer is synthesized by ceramide galactosyltransferase (CGalT) at the lumen of the endoplasmic reticulum (ER). These enzymes do not share any sequence similarity and CGlcT possesses its transmembrane region at the N-terminal (type III) whereas CGalT possesses it at the C-terminal (type I).<ref>Sprong H, Degroote S, Nilsson T, Kawakita M, Ishida N, van der Sluijs P, van Meer G "Association of the Golgi UDP-galactose transporter with UDP-galactose:ceramide galactosyltransferase allows UDP-galactose import in the endoplasmic reticulum" Mol Biol Cell. 2003;4:3482-93　PMID 12925779</ref>
|
哺乳動物では、ゴルジ装置の細胞質側に局在するグルコシルセラミド合成酵素（CGlcT）によって GlcCer が、小胞体の内腔側に局在するガラクトシルセラミド合成酵素（CGalT）によって GalCer が作られます。これらの酵素は配列相同性が無く、CGlcT は膜貫通領域が N 末端側（III型酵素）、CGalT は C 末端側（I型酵素）です。
}}
}}
{| class="wikitable" style="text-align:center"
|-
!
! GlcCer || GalCer
|-
! Enzyme
| CGlcT (EC 2.4.1.80)
| CGalT (EC 2.4.1.45)
|-
! Reaction
| Ceramide + UDP-glucose &rarr; GlcCer + UDP
| Ceramide + UDP-galactose &rarr; GalCer + UDP
|-
! Location
| '''Golgi (outside)''' &rarr; flipflop &rarr; Golgi (inside)
| '''ER lumen'''
|}
{{Twocolumn|
{{Twocolumn|
<big>GalCer</big> has been observed in Eubacterial Domain, but not in Protozoa, Plant, or Fungi (except Ascomycota). It is widely observed in Animal Kingdom, but not in Ecdysozoa (cuticle-shedding animals) of Protostomia. Its ceramide structure differs widely as in GlcCer but not so complex as GlcCer in Plant and Fungi. GalCer is the basic structure for sulfatide (3-O-sulfogalactosylceramide or SM4) and Gala Series often found in Annelida (segmented worm). In Mammalia (of Deuterostomia), GalCer is major in the nerve tissue, especially myelin sheath, whose amido-linked fatty acids are hydroxylated at the C-2 position for more than 50%. Krabbe disease (globoid cell leukodystrophy) is an inborn metabolic disorder in which a small amount of psychosine (galactosylsphingosine, i.e., the lyso form of GalCer) induces demyelination because of the deficiency of the enzyme galactosylceramidase <ref>GalCer accumulates in the kidney and not in nerve tissues. See Igisu et al. (1983) Biochem Biophys Res Commun 110:940-944; Igisu et al. (1983) Brain 106:405-417;Igisu & Suzuki (1984) J Neuropath Exp Neurol 43:22-36.</ref>. &alpha;-GalCer, found from Porifera (marine sponge), in which galactose is &alpha;-bonded to ceramide (not the usual &beta;-bond), uniquely binds to human or mouse NKT cells and is considered a potent anti-tumor agent.
Since all glycolipid synthesizing enzymes (except for CGlcT and CGalT) reside at the lumen of the Golgi apparatus, GlcCer must be flip-flopped from the cytosolic side to the lumen by an eyzyme called flippase.
To synthesize GalCer, UDP-galactose and ceramide must transport the ER membrane. UDP-galactose transporter 2 (UGT2) is considered to be responsible.
 
GalT can also work on glycerolipid, and synthsizes sulfated glyceroglycolipid in mammalian testis, called seminolipid.
|
|
'''GalCer'''は真正細菌には存在するが、原生生物界、植物界、菌界（子嚢菌を除く）には報告がない。また動物界において広く分布するが旧口動物である脱皮動物には確認されていない。セラミドはGlcCerと同様にその構造は多岐にわたるが植物や菌類のGlcCerほど複雑ではない。GalCerはスルファチド (3-O-硫酸化ガラクトシルセラミドまたはSM4) や環形動物門に多く含まれるガラ系列の基本構造になる。新口動物である哺乳綱では神経系、特にミエリン鞘にGalCerが多く含まれ、その脂肪酸の50%以上は、C2位が水酸化されている。クラッベ病（グロボイド細胞ロイコジストロフィー; GLD）はガラクトシルセラミダーゼの欠損により微量のサイコシン（ガラクトシルスフィンゴシン、つまり GalCer のリゾ体）が蓄積して脱髄（ミエリンの破壊）を起こす遺伝性疾患である。また、海綿動物門に発見された&alpha;-GalCerは、ガラクトースがセラミドに（通常の&beta;でなく）&alpha;結合しており、ヒトやマウスのNKT細胞に特異的に結合することにより抗腫瘍効果を期待されている。
CGlcT, CGalT 以外の糖脂質合成酵素はゴルジ装置の内腔側にあり、GlcCer はゴルジ膜の細胞質側から内腔側にフリッパーゼと呼ばれる酵素で移動（フリップフロップ）します。GalCer の場合は、UDPガラクトースとセラミドが小胞体膜を通過します。UDPガラクトーストランスポータ2 (UGT2) が関与するようです。
 
CGalTはグリセロ脂質にも作用し、精巣の硫酸化グリセロ糖脂質であるセミノリピドも合成します。
}}
}}


<references/>
<references/>

Latest revision as of 05:12, 18 May 2023

Cerebroside


Upper classes: LB LBS

| OH
(C14-chain)＝／＼／ーO-Glucose
(fatty acid) ー | NH
GlcCer
| OH
(C14-chain)＝／＼／ーO-Galactose
(fatty acid) ー | NH
GalCer

Monoglycosylceramides (or cerebrosides) are glycosphingolipids where a single sugar is attached to the ceramide structure. Especially, glucopyranose-attached Glucosylceramide (GlcCer) and galactopyranose-attached Galactosylceramide (GalCer) are widely observed in all kingdoms. GlcCer and GalCer are not registered in LipidBank. Instead, we summarize their distribution with bibliographic references with species information.

モノグリコシルセラミド (またはセレブロシド) はセラミドに糖が1個エステル結合したスフィンゴ糖脂質です。特にグルコース（ピラノース型）がβ結合したグルコシルセラミド (GlcCer) とガラクトース（ピラノース型）がβ結合したガラクトシルセラミド (GalCer) は生物界に広く分布します。データベース中には掲載せず、ここに解説と生物種ごとの報告例を掲載します。

Glucosylceramide (GlcCer)

GlcCer is widely observed not only in Animal, Plant or Fungi kingdoms, but also in Protozoa Kingdom[1]. Ceramide structures (hydrocarbon chain length, the number and position of double bonds, existence of hydroxy and other modification groups) may vary, and become complex especially in Plant and Fungi Kingdoms, and Protostomia [2]. In Animal Kingdom, GlcCer is the basic structure for different derivative series such as Globo, Lacto, and Ganglio Series. In Protostomia, GlcCer is the major ceramide in its nerve tissue, e.g. shrimp brain [3], and its amide-linked fatty acids do not contain hydroxy groups. GlcCer in plants contains many α-hydroxy fatty acids. In some cold-resistant plants ω9-unsaturated fatty acids are detected. In Mammalia (of Deuterostomia), GlcCer is major in the spleen, blood, and skin.

Gaucher's disease is an inborn metabolic disorder in which GlcCer accumulates in the liver and spleen because of the deficiency of the enzyme glucocerebrosidase to dissociate glucose from the ceramide structure [4].

GlcCerは系統発生的にみると動物の各組織に広く分布するだけでなく、植物界や菌界、原生生物界にも含まれます。セラミド構造（炭化水素の鎖長、二重結合の数と位置、水酸基の有無、修飾基の有無など）の組み合わせは多岐にわたり、特に植物界や菌界、旧口動物のセラミドは複雑です。動物界において、GlcCerはさまざまな糖鎖を持つ糖脂質の基本構造になり、グロボ系列、ラクト系列、ガングリオ系列などが生成されます。旧口（前口）動物の神経系にはGlcCerが多く含まれ（例えばエビ）、セラミド骨格の脂肪酸に水酸基を含みません。対して、植物は α-水酸化の脂肪酸が多く、耐冷性植物の一部からは ω9-不飽和の脂肪酸も見いだされます。後口動物の哺乳綱において、GlcCerは、脾臓、血液、皮膚などに多く含まれます。

先天性代謝異常症であるゴーシェ (Gaucher) 病は、GlcCerを分解するグルコセレブロシダーゼが欠損し、肝臓や脾臓にGlcCerが蓄積して発症します。

Galactosylceramide (GalCer)

GalCer has been observed in Eubacterial Domain, but not in Protozoa, Plant, or Fungi (except Ascomycota). It is widely observed in Animal Kingdom, but not in Ecdysozoa (cuticle-shedding animals) of Protostomia. Its ceramide structure differs widely as in GlcCer but not so complex as GlcCer in Plant and Fungi. GalCer is the basic structure for sulfatide (3-O-sulfogalactosylceramide or SM4) and Gala Series often found in Annelida (segmented worm). In Mammalia (of Deuterostomia), GalCer is major in the nerve tissue, especially myelin sheath, whose amido-linked fatty acids are hydroxylated at the C-2 position for more than 50%.

Krabbe disease (globoid cell leukodystrophy) is an inborn metabolic disorder in which a small amount of psychosine (galactosylsphingosine, i.e., the lyso form of GalCer) induces demyelination because of the deficiency of the enzyme galactosylceramidase [5]. α-GalCer, found from Porifera (marine sponge), in which galactose is α-bonded to ceramide (not the usual β-bond), uniquely binds to human or mouse NKT cells and is considered a potent anti-tumor agent.[6] Recently, α-linked GlcCer and GalCer were reported in NKT cells in trace amount[7].

GalCerは真正細菌に存在しますが、原生生物界、植物界、菌界（子嚢菌を除く）では報告がありません。動物界に広く分布しますが旧口動物である脱皮動物には確認されていません。セラミド構造は、GlcCerと同様に多様ですが、植物や菌類のGlcCerほど複雑ではありません。GalCerはスルファチド (3-O-硫酸化ガラクトシルセラミドまたはSM4) や環形動物門に多く含まれるガラ系列の基本構造です。 後口動物である哺乳綱では神経系、特にミエリン鞘にGalCerが多く含まれ、その脂肪酸の50%以上は、C2位が水酸化されています。

クラッベ病（グロボイド細胞ロイコジストロフィー; GLD）はガラクトシルセラミダーゼの欠損により微量のサイコシン（ガラクトシルスフィンゴシン、つまり GalCer のリゾ体）が蓄積して脱髄（ミエリンの破壊）を起こす遺伝性疾患です。また、海綿動物門に発見されたα-GalCerは、ガラクトースがセラミドに（通常のβでなく）α結合しており、ヒトやマウスのナチュラルキラーT (NKT) 細胞に特異的に結合し抗腫瘍効果がみられます。α結合型の GlcCer および GalCer はNKT細胞に微量に存在します。

  1. ↑ Tan RX, Chen JH. "The cerebrosides" Nat Prod Rep. 2003;20:509-34.PMID 14620845, Yu R.K. Yanagisawa M. Ariga T. Glycosphingolipid Structures,in Comprehensive Glycoscience Vol.1,pp. 73-122,doi 10.1016/B978-044451967-2/00003-9, Itonori S, Sugita M. Glycophylogenetic Aspects of Lower Animals. in Comprehensive Glycoscience Vol.3, pp. 253-84, doi 10.1016/B978-044451967-2/00050-7 (J.P. Kamerling, ed.) Elsevier, Oxford (UK). (2007)
  2. ↑ Sphingoid base chain may terminate with an isopropyl group in spongia, annelida, echinodermata, and bacteria. Terminal isobutyl or sec-pentyl group has been found only in echinodermata. Methylated or cyclopropane-containing sphingoid base is found in spongia, echinodermata, protochordata, and fungi.
  3. ↑ Okamura N, Stoskopf M, Hendricks F, Kishimoto Y. "Phylogenetic dichotomy of nerve glycosphingolipids" Proc Natl Acad Sci, 1985 82:6779-6782 PMID 3863128
  4. ↑ Brady RO, Kanfer JN, Shapiro D. "Metabolism of glucocerebrosides. II. Evidence of an enzymatic deficiency in Gaucher's desease" Biochem Biophys Res Commun. 1965;18:221-5 PMID 14282020
  5. ↑ Suzuki K. "Globoid cell leukodystrophy (Krabbe's disease): update" J Child Neurol. 2003;18:595-603.PMID 14572137, Suzuki K. "Evolving concept of the pathogenesis of globoid cell leukodystrophy (Krabbe disease)" Proc Jpn Acad Ser B Phys Biol Sci 2003; 79:1-8
  6. ↑ Kitamura H, Iwakabe K, Yahata T, Nishimura S, Ohta A, Ohmi Y, Sato M, Takeda K, Okumura K, Van Kaer L, Kawano T, Taniguchi M, Nishimura T. "The natural killer T (NKT) cell ligand alpha-galactosylceramide demonstrates its immunopotentiating effect by inducing interleukin (IL)-12 production by dendritic cells and IL-12 receptor expression on NKT cells" J Exp Med. 1999; 5;189(7):1121-8. PMID 10190903
  7. ↑ Kain L et al. "The Identification of the Endogenous Ligands of Natural Killer T Cells Reveals the Presence of Mammalian α-Linked Glycosylceramides" Immunity 41(4), 543-554, 2014 PMID 25367571

Cerebrosides in Mammals

Lipids in Heart Muscle

Lipids in Rat Testis

Cerebroside and Sulfatide Ratio in Animal Brain

Fatty acid composition of bovine brains

Sphingolipids in All Kingdoms

The table lists biological species from which sphingolipids were identified. Icons indicate representative species. The icons are used in sphingolipid references together with Synthesis.png for chemical synthesis.

ここに示す分類表にはスフィンゴ脂質が報告された生物が記載されており、アイコンは代表種を表します。スフィンゴ脂質関連の文献にはこれらのアイコンの他、化学合成を表すSynthesis.pngも使われます。

Domain Kingdom Phylum Class Order Model organism Icon Galactosylceramides (GalCer)
(*β configuration unless otherwise state) 		Glucosylceramides (GlcCer)
(*β configuration unless otherwise state) 		Glycosphingolipids
(GSL) 		Ceramide phosphoethanolamine
(CPE) 		Sphingomyelin
(SM) 		Ceramide aminoethylphosphonate
(CAEP) 		Ceramide methylaminoethylphosphonate
(CMAEP) 		Glycosyl inositol phosphoceramides
(GIPC)
ドメイン 界 門 綱 目 モデル生物 Species
(Structure) 		FA, LCB References Species
(Structure) 		FA, LCB References Species
(Structure) 		FA, LCB References Major series Species
(Structure) 		FA, LCB References Species
(Structure) 		FA, LCB References Species
(Structure) 		FA, LCB References Species
(Structure) 		FA, LCB References Species
(Structure) 		FA, LCB References
真正細菌
Bacteria 		バクテロイデス門
Bacteroidetes 		腸内細菌 Bacteria.png Bacteroides
fragilis
(α-GalCer) 		br-h17:0,
br-d17:0 		PubMed Bacteroides
fragilis 		br-h17:0,
br-d17:0 		PubMed Sphingobacterium
spiritivorum
/ATCC33861
(Ins-P-Cer) 		br-15:0,
br-h15:0,
br-d17:0 		PubMed
シアノバクテリア門
Cyanobacteria 		藍色細菌 Scytonema
julianum
(Acetyl-
sphingomyelin) 		15:1,d18:1 PubMed
プロテオバクテリア門
Proteobacteria 		大腸菌 Cystobacter
fuscus
(β-GalCer) 		br-h17:0,
br-d19:2 		DOI Sphingomonas
paucimobilis
(GlcAα-Cer) 		h14:0,d18:0 PubMed Sorangium
cellulosum 		h16:1,
br-h17:1,
h18:1,
br-d21:3,
br-d21:2 		PubMed Bacteriovorax
stolpii 		br-h15:0,
br-15:0,
br-t17:0,
br-d17:0 		PubMed
真核生物
Eukaryota 		原生生物界
Protista 		メタモナーダ門 Metamonada 鞭毛虫 Protista.png Giardia
lamblia 		undescribed PubMed Trichomonas
vaginalis 		16:0,d18:1 PubMed Giardia
lamblia 		16:0,d18:1 DOI
ユーグレノゾア門
Euglenozoa 		Leishmania
amazonensis

Plasmodium
falciparum 		undescribed

h10:0,h120,
d18:0,d20:0 		PubMed

PubMed 		Leishmania
amazonensis
(Galβ1-3Galα1
-4Galβ1
-4Glcβ1-Cer)

Plasmodium
falciparum
(trihexosyl-
ceramide)

Trypanosoma
brucei
(GM3,GM1,
GD1a,GD1b) 		undescribed

h12:0,h14:0,
d18:0,d20:0

undescribed 		PubMed

PubMed

PubMed 		Globo,
a-series,
b-series 		Leishmania
major 		18:0,d16:1 PubMed Plasmodium
falciparum 		undescribed PubMed Trypanosoma
brucei
(Ins-P-Cer) 		16:0,17:0,
18:0 d16:0,
d17:0,d18:1,
d18:0,d19:0,
d20:0,d20:1 		PubMed
(*Procyclic
formのみ)
繊毛虫門
Ciliophora 		繊毛虫 Entodinium
caudatum 		undescribed PubMed Tetrahymena
pyriformis
/WH-14 		16:0,
br-17:0,
br-18:0,
br-h17:0,
h18:0,
br-d17:1,
br-d18:1 		PubMed Tetrahymena
pyriformis
/WH-14 		16:0,
br-18:0,
br-h17:0,
d18:1 		PubMed
植物界
Plantae 		緑藻植物門
Chlorophyta 		クロレラ Plantae.png Pseudococcomyxa
chodatii 		h16:0,h18:0,h20:0,
h22:0,h24:0
d18:2,t18:0,t18:1 		DOI Chlorella
kessleri
(Galα1-4Galβ1
-4Glcβ1-Cer) 		undescribed PubMed Globo Chlorella
variabilis
(Hex(2)-Ins
-P-Cer) 		h24:1, t18:0 Nebraska
Univ.,2015
(*学位論文。
ESI-MS分析,
糖の種類は
Hexとしか
判別できない)
ストレプト植物門
Streptophyta
(*被子植物門を除く) (except Angiosperm) 		緑色植物 Ginkgo
biloba

Stenochlaena
palustris 		h16:0,d18:2

h24:0,d18:2 		DOI

DOI 		Pteridum
aquilinum
(HexNAc-HexA
-(Hex-)Ins
-P-Cer) 		h24:0,t18:0 PubMed
(*MALDI-MS分析,
糖の種類は
Hexとしか
判別できない)
被子植物門
Angiosperm 		シロイヌナズナ Arabidopsis
thaliana 		16:0,h24:1,
d18:1,t18:1 		PubMed Prunus
armeniaca
/kernel 		undescribed J.Chromatogra.,
294,519-524,
1984 		Arabidopsis
thaliana
/leaves
(Glc-GlcA
-Ins-P-Cer) 		h24:0,t18:1 PubMed
菌界
Fungi 		接合菌門
Zygomycota 		ケカビ、クモノスカビ Fungi.png Mucor
hiemalis 		h14:0,h16:0,
br-d19:2,d20:1 		PubMed Mucor
hiemalis
(Galβ1-6Galβ1
-Cer) 		h24:0,h25:0,
h26:0,t18:0 		PubMed Gala PubMed
(*接合菌門に
はGIPC
は含まれ
ない。)
子嚢菌門
Ascomycota 		酵母、コウジカビ Aspergillus
oryzae 		h18:0,br-d19:2 PubMed Aspergillus
oryzae 		h18:0,br-d19:2 PubMed Neurospora
crassa
(Glcα1-2Galβ1-
6Galβ1-6Galβ1-Cer) 		h24:0,t18:0 PubMed Gala Aspergillus
nidulans
(Manα1-3Manα
-2Ins-P-Cer) 		h24:0,t18:0 PubMed
担子菌門
Basidiomycota 		キノコ Cryptococcus
neoformans 		h18:0,br-d19:2 PubMed Cryptococcus
neoformans
(Ins-P-6Manα1
-2Ins-P-Cer) 		undescribed PubMed
動物界
Animalia 		海綿動物門
Porifera 		カイメン Porifera.png Agelas
mauritianus
(α-GalCer)


Chondropsis sp.
(β-GalCer)

h24:0,br-t18:0


h24:0,br-d18:1

DOI


DOI

Haliclona sp. h22:0,t18:1 DOI Amphimedon
viridis
(GlcNAcα1-Cer,
GlcNAcβ1-Cer) 		h22:0,br-t20:1,
h22:0,br-t20:1,
br-t19:0 		DOI Svenzea
zeai
(Arapβ1
-6Ins-P-Cer) 		16:0,
br-16:0,
br-17:0,
d26:1,d26:0 		PubMed
刺胞動物門
Cnidaria 		クラゲ Cnidaria.png Metridium
senile

Sarcophyton ehrenbergi
(α-GlcCer) 		h16:0,h20:0,
br-d19:2

h18:0,br-d19:3 		PubMed

PubMed 		PubMed
(*刺胞動物門はCPEをもたない。) 		PubMed
(*刺胞動物門はSMをもたない。) 		Aurelia
aurita 		14:0,16:0,
d18:1,t18:0 		PubMed Aurelia
aurita 		14:0,16:0,
d18:1,t18:0 		PubMed
    (旧口動物、冠輪)
(Protostomia, Lophotrochozoa) 		扁形動物門
Platyhelminthes 		吸虫綱
Trematoda 		ジストマ Platyhelminthes.png Fasciola
hepatica 		18:0,h18:0,
t18:0,t20:0 		PubMed Fasciola
hepatica

Schistosoma mansoni 		18:0,h18:0,
t18:0,t20:0

h16:0,t18:0,
t20:0 		PubMed

PubMed 		Fasciola
hepatica
(Galα1-4Galβ1
-4-Glcβ1-Cer)

Schistosoma
mansoni
(GalNAcβ1
-4Glcβ1-Cer)

Schistosoma
mansoni
(GM1,GD1a,
GD1b,GT1b) 		undescribed

h16:0,26:0

undescribed 		PubMed

PubMed

DOI 		Globo,
Schisto,
Ganglio,
a-series 		Paramphistomum
cervi 		16:0,18:1 PubMed
渦虫綱
Tubellaria 		プラナリア Dugesia
dorotocephala 		undescribed PubMed
条虫綱
Cestoda 		サナダムシ Diphyllobothrium
hottai 		16:0,18:0,26:0,
28:0,d18:0,
d20:0,t18:0,
t20:0 		PubMed Diphyllobothrium
hottai 		16:0,18:0,
26:0,28:0,
d18:0,d20:0,
t18:0,t20:0 		PubMed Diphyllobothrium
hottai
(Galβ1-4(Fucα1-3)Glcβ1-3Galβ1-Cer)

Spirometra
erinacei
(Galβ1-4(Fucα1-3)Glcβ1-3Galβ1-Cer)

Echinococcus
multilocularis
(GM1,GM3,
GD1a,GM2) 		16:0,18:0,
26:0,28:0,
d18:0,d20:0,
t18:0

18:0,18:1,
d18:0,t18:0

16:0,24:0,
24:1,d18:1 		PubMed

PubMed

PubMed 		Spirometo,
a-series 		Hymenolepis
diminuta 		undescribed PubMed
外肛動物門
Bryozoa 		コケムシ Pectinatella
magnifica 		undescribed DOI Pectinatella
magnifica
(GlcNAcβ1
-4Glcβ1-Cer) 		undescribed DOI
腕足動物門
Brachiopoda 		シャミセンガイ Brachiopoda.png Lingula
unguis 		h16:0,h17:0,
h18:0,d18:1,
d18:3 		DOI Lingula
unguis
(Manα1-3Manβ1
-4Glcβ1-Cer) 		16:0,18:0,
d18:3 		DOI Mollu Lingula
unguis 		16:0,18:0,
h16:0,h18:0,
d18:1,d18:3 		滋賀大学教育学部紀要,自然科学・教育科学,45,31-42,1995
環形動物門
Annelida 		貧毛綱
Oligochaeta 		ミミズ Annelida.png Pheretima
aspergillum 		22:0,24:0,
h22:0,h23:0,
h24:0,br-d18:1,
d18:1,t18:0 		DOI Pheretima
aspergillum 		22:0,24:0,
br-d18:1,
d18:1,
br-d19:1 		DOI Pheretima
aspergillum
(Galβ1-6Galβ1-Cer)

Pheretima
hilgendorfi
(PC-6Galβ1
-6Galβ1-Cer) 		22:0,24:0,
h24:0,
br-d18:1,
d18:1,t18:0

22:0,24:0,
br-d18:1,
br-d19:1 		DOI

PubMed 		Neogala (*環形動物門では
SMは見つからない。)
PubMed
(*SM-Binding Protein, Lyseninはミミズから抽出。)
ヒル綱
Hirudinea 		ヒル Hirudo
nipponica
(β-GalCer) 		16:0,22:0,
24:0,d18:1,
br-d19:1,
d22:3 		DOI Hirudo
nipponica
(Galα1-6Galβ1-Cer,
PC-6Galβ1
-6Galβ1-Cer) 		16:0,22:0,
24:0,d18:1,
br-d19:1,
16:0,22:0,
24:0,t18:0,
br-t19:0,
d22:3 		DOI Isoneogala (*環形動物門ではSMは見つからない。)
多毛綱
Polychaeta 		サシバゴカイ目
Phyllodocida 		イトメ Tylorrhynchus
heterochaetus 		16:0,17:0,
18:0,h16:0,
h17:0 d18:1,
d18:2 		DOI Tylorrhynchus
heterochaetus 		16:0,17:0,
18:0,h16:0,
h17:0 		DOI Tylorrhynchus
heterochaetus
(PC-6Galβ1-Cer) 		16:0,d18:1,
d18:2 		DOI (*環形動物門ではSMは見つからない。) Tylorrhynchus
heterochaetus
(InsMe-P-Cer) 		h16:0,h18:0,
t18:0 		DOI
ケヤリ目
Sabellida 		エラコ Pseudopotamilla
occelata 		16:0,18:0,
h16:0,h18:0,
d18:1 		DOI Pseudopotamilla
occelata 		16:0,18:0,
h16:0,h18:0,
d18:1 		DOI Pseudopotamilla
occelata
(Galα1-4Galβ1-Cer,
Galβ1-4Glcβ1-Cer) 		16:0,18:0,
d18:1,
20:1,22:1,
d18:1 		DOI Gala (*環形動物門ではSMは見つからない。) Pseudopotamilla
occelata
(Ins-P-Cer) 		undescribed DOI
軟体動物門
Mollusca 		二枚貝綱
Bivalvia 		二枚貝 Mollusca.png Hyriopsis
schlegelii
/egg 		14:0,16:0,
h16:0,d18:1,
br-d19:1 		PubMed Hyriopsis
schlegelii
/egg 		14:0,16:0,
h16:0,d18:1,
br-d19:1 		PubMed Hyriopsis
schlegelii
/sperm
(Manα1-3Manβ1
-4Glcβ1-Cer) 		16:0,18:0,
20:0,d18:1,
br-d18:1 		PubMed
PubMed 		Mollu Pinctada
martensii 		h16:0,h18:0,
d18:1,d18:0 		PubMed Pinctada
martensii 		16:0,17:0,
18:0,d16:1,
d18:1,d18:2 		滋賀大学教育学部紀要,自然科学,54,41-48,2004 Hyriopsis
schlegelii
/egg 		15:0,16:0,
d18:1 		DOI Mytilus
galloprovincialis 		16:0,h16:0,
d18:1,t18:0 		PubMed
腹足綱
Gastropoda 		巻貝 Chlorostoma
argyrostoma
turbinatum 		16:0,h16:0,
h18:0,d18:1,
d18:2 		PubMed Euhadra
hickonis 		16:0,h16:0,
br-d19:1,
d18:1 		DOI Chlorostoma
argyrostoma
turbinatum
(Galβ1-6Galβ1-Cer) 		16:0,17:0,
h16:0,h18:0,
d18:1,
br-d18:1,
d18:2,d19:1 		PubMed Neogala Heterogen
longispira 		undescribed PubMed Sinotaia
historica 		undescribed PubMed Monodonta
labio 		undescribed PubMed Monodonta
labio 		undescribed PubMed
頭足綱
Cephalopoda 		イカ、タコ Ommastrephes
sloani pacificus
/sperm 		16:0,18:0,
22:0,22:1,
d16:1 		滋賀大学教育学部紀要,自然科学・教育科学,44,17-23,1994 Ommastrephes
sloani pacificus
/sperm 		16:0,18:0,
22:0,22:1,
d16:1 		滋賀大学教育学部紀要,自然科学・教育科学,44,17-23,1994 Ommastrephes
sloani pacificus
/sperm
(Manβ1-4Manβ1
-4Glcβ1-Cer)

Todarodes
pacificus
(GT3,GQ1c) 		16:0,18:1,
22:1,24:1,
d16:1

undescribed 		滋賀大学教育学部紀要,自然科学・教育科学,44,17-23,1994

PubMed 		c-series Todarodes
pacificu
/viscera 		16:0,18:0,
24:1,d16:1 		滋賀大学教育学部紀要,自然科学,59,29-37,2009 Todarodes
pacificus
/viscera 		16:0,22:1,
24:1,d16:1 		滋賀大学教育学部紀要,自然科学,59,29-37,2009
(旧口動物、脱皮)
(Protostomia, Ecdysozoa) 		線形動物門
Nematoda 		クロマドラ綱
Chromadorea 		回虫 Nematoda.png Ascaris
suum
/adult

Ascaris
suum 		h24:0,br-d17:1,
br-d17:0,
br-t17:0,t18:0 		PubMed

DOI 		Ascaris
suum
(HSO3-3Galβ1-Cer)

Ascaris
suum
/adult
(GlcNAcβ1-3Manβ1
-4Glcβ1-Cer)

Ascaris
suum
(PC-6GlcNAcβ1-3Manβ1-4Glcβ1-Cer) 		24:0,h24:0,br-d17:1,
br-d17:0,
br-t18:0

h24:0,br-d17:1,
br-d17:0

h22:0,h24:0,
br-d17:1,
br-d17:0 		PubMed

PubMed

DOI 		Arthro Ascaris
suum 		24:0,h24:0,
br-d17:1,
br-d17:0,
br-t17:0 		DOI Ascaris
suum
(Galα1-
2Ins-P-Cer) 		24:0,h24:0,
br-d17:0,
d18:0 		PubMed
節足動物門 Arthropoda 昆虫綱 Insecta　 ハエ Arthropoda.png Lucilia
caesar
/larvae 		16:0,18:0,
20:0,22:0,
d14:1,d16:1 		PubMed Lucilia
caesar
/larvae
(GlcNAcβ1-3Manβ1
-4Glcβ1-Cer) 		16:0,18:0,
20:0,22:0,
d14:1,d16:1 		PubMed Arthro Lucilia
caesar
/pupae 		20:0,22:0 PubMed
(*昆虫綱ではショウジョウバエなど少数の種のみCPEを多くもつ。) 		Aedes
aegypti
/cells 		18:0,20:0,
22:0 		PubMed
(*昆虫綱では多くの種がSMをもつ。)
甲殻亜門
軟甲綱
Crustacea
Malacostraca 		エビ、カニ Penaeus
duorarum
/ventral nerve 		undescribed PubMed Euphausia
superba
(GlcNAcβ1-3Manβ1
-4Glcβ1-Cer)

Euphausia
superba
(MAEPn-6Glcβ1-Cer) 		22:1,24:1,
d14:1

22:1,h22:1,
h24:1,d14:1,
br-d18:3,
d18:3 		DOI

PubMed 		Arthro Erimacrus
isenbeckii 		18:0,20:0,
22:0,22:1,
d14:1,d14:0 		滋賀大学教育学部紀要,自然科学,52,9-16,2002
甲殻亜門
鰓脚綱
Crustacea　Branchiopoda 		カブトエビ、ミジンコ Artemia
franciscana 		18:0,22:0,
h18:0,d16:1,
d17:1 		PubMed Artemia
franciscana
(GlcNAcβ1-3Manβ1
-4Glcβ1-Cer) 		22:0,d16:1,
d17:1 		PubMed Arthro Artemia
franciscana 		18:0,22:0,
d16:1,d17:1 		PubMed
多足亜門
ヤスデ綱
Myriapoda Diplopoda 		ヤスデ Parafontaria
laminata 		h(2- or 3-hydroxy)23:0,
h(2- or 3-hydroxy)24:0,
d17:1,br-d18:1,
d18:1 		PubMed Parafontaria
laminata
(GlcNAcβ1-3Manβ1
-4Glcβ1-Cer) 		22:0,23:0,
24:0,d17:1,
br-d18:1 		PubMed Arthro Parafontaria
laminata 		16:0,17:0,
18:0,22:0,
d17:1,d18:1 		滋賀文化短期大学紀要,3,71-78,1993 Spirostreptus
asthene s
/oocytes 		undescribed DOI
(新口動物)
(Deuterostomia) 		棘皮動物門
Echinodermata 		ウミユリ綱
Crinoidea 		　 ウミユリ Echinodermata.png Comanthus
japonica 		24:0,h18:0,
h22:1,h24:0,
d16:1,d18:2,
t16:0 		PubMed Comanthus
japonica
(Ins-P-Cer) 		22:0,24:0,
d16:1 		DOI
DOI
(*9-O-MeNeuGc
が結合したGIPC
がある。)
DOI
(*9-O-MeNeuAc
が結合したGIPC
がある。)
ヒトデ綱
Asteroidea 		ヒトデ Culcita
novaeguineae 		h22:0,br-t16:0 PubMed Asterias
rubens 		d18:2,d22:1,
d22:2 		PubMed Luidia
maculata
(Galβ1-4Glcβ1-Cer)

Luidia
maculata
((3-sulfo)Galβ1
-4Galβ1
-4Glcβ1-Cer)

Luidia
maculata
(GM3)

Luidia
maculata
(GD3) 		h16:0,h22:0,
h24:0,
br-d19:1,
t16:0,
br-t17:0,
t22:1

h22:0,
br-t19:0

h22:0,t19:0

h22:0,t17:0 		DOI

DOI

DOI

PubMed 		a-series,
b-series 		Distolasterias
nipon 		undescribed DOI
クモヒトデ綱
Ophiuroidea 		クモヒトデ Ophiocoma
scolopendrina
(NeuGcα2
-6Glcβ1-Cer,
GM5,
HSO3-8NeuAcα2
-6Glcβ1-Cer) 		18:0,t18:0,
br-t18:0,
h22:0,t18:0,
br-t18:0 		PubMed Echino
ウニ綱
Echinoidea 		ウニ Anthocidaris
crassispina
/egg 		22:1,24:1,
h22:1,h24:1,
t18:0 		PubMed Anthocidaris
crassispina
/egg
(Galα1-6Glcβ1Cer)

Anthocidaris
crassispina
/egg
(NeuGcα2
-6Glcβ1-Cer,
GM5,
HSO3-8NeuGcα2
-6Glcβ1-Cer) 		22:1,24:1,
h22:1,h24:1,
t18:0

h22:1,h23:1,
h24:1,t18:0 		PubMed

PubMed 		Echino Strongylocentrotus
intermedius 		undescribed DOI
ナマコ綱
Holothuroidea 		ナマコ Bohadschia
argus 		h24:1,d17:1,
br-d17:1 		PubMed Cucumaria
echinata 		h22:0,h23:0,
h24:0,
br-d17:1 		DOI Holothuria
leucospilota
(NeuGcα2
-6Glcβ1-Cer,
GM5) 		h22:0,h23:0,
h24:0,h24:1,
br-t17:0,br-t18:0,
br-t19:0 		DOI Echino
脊索動物門
Chordata 		ホヤ綱
Ascidiacea 		ホヤ Ascidia.png Aplidium
nordmani,
Styela
partita,
Botryllus
leachii 		h24:0,t18:0,
br-t19:0 		DOI Microcosmus
sulcatus
(Galβ1-4(Fucα1
-3)Glcβ1-Cer) 		h22:0,h23:0,
t19:0 		DOI Ciona
intestinalis 		16:0,18:0,
18:1,d16:1,
d18:2 		PubMed
軟骨魚綱
Chondrichthyes 		エイ Chondrichthyes.png Carcharhinus
plumbeus
/brain
(Gal-Cer,SO3-3Galβ1-Cer) 		undescribed PubMed Squalus
acanthias
/rectal gland 		undescribed PubMed Bathyraja
smirnovi
(GM2,GD2) 		undescribed PubMed a-series,
b-series 		Torpedo
marmorata
/electric organ 		16:0,24:1,
d18:1 		PubMed
条鰭綱
Actinopterygii 		硬骨魚 Actinopterygii.png Cyprinus
carpio
/brain
(Gal-Cer,SO3-3Galβ1-Cer) 		24:1,h24:0 PubMed Theragra
chalcogramm
/brain 		24:1 PubMed Latimeria
chalumnae
(GM3,GD3)

Theragra
chalcogramma
(9-O-Ac-GT3) 		16:0,18:0,
24:1,d18:1

undescribed 		DOI

PubMed 		a-series,
b-series,
c-series 		Latimeria
chalumnae
/brain 		undescribed DOI
両生綱
Amphibia 		カエル Amphibia.png Rana
catesbeiana
/brain
(Gal-Cer,SO3-3Galβ1-Cer) 		18:0,24:1,
h24:1,d18:0,
d18:1 		PubMed Bufo
japonicus
/skin 		22:0,24:0,
h22:0,h24:0,
t18:0 		PubMed Rana
pipiens
(GD1b,GT1b) 		undescribed DOI b-series Rana
pipiens
/spinal cord 		undescribed J.Exp.Biol. 29, 203-210, 1952
爬虫綱
Reptilias 		ワニ Reptilia.png Iguana spp.
/brain
(Gal-Cer,SO3-3Galβ1-Cer) 		nonhydroxy FA,hydoroxy FA PubMed Anolis
carolinensis
/kidney 		h23:0,h24:0,
h24:1,d18:1 		PubMed Pseudemys
scripta elegans
/brain
(GM1,GD1a,GD1b,
GT1b,GQ1b) 		undescribed PubMed a-series,
b-series 		Tropidurus
torquatus 		undescribed PubMed
鳥綱
Aves 		トリ Aves.png Gallus
gallus domesticus
/sciatic nerve
(Gal-Cer,SO3-3Galβ1-Cer) 		22:0,24:0,
h22:0,h24:0 		PubMed Gallus
gallus
domesticus
/lens 		undescribed PubMed Anser
anser
/brain
(GM1,GD1a,GD1b,
GT1b,GQ1b) 		undescribed PubMed a-series,
b-series 		Colurnba
livia domestica
/liver 		16:0,18:0,
20:4 		PubMed
哺乳綱
Mammalia 		無盲腸目
Eulipotyphla 		モグラ Mammalia.png Suncus
murinus
/brain
(Gal-Cer,SO3-3Galβ1-Cer) 		24:0,h24:0,
d18:1 		PubMed Suncus
murinus
/brain 		24:0,h24:0,
d18:1 		PubMed Suncus
murinus
/brain
(GalNAcβ1-4Galβ1
-4Glcβ1-Cer,
GM1,GD1a,GD1b,
GT1b) 		24:0,d18:1 PubMed Ganglio,
a-series,
b-series 		Suncus
murinus
/liver 		undescribed PubMed
食肉目
Carnivora 		ネコ、イヌ Felis
silvestris catus
/brain
(Gal-Cer,SO3-3Galβ1-Cer) 		undescribed PubMed Canis
lupus familiaris
/intestine 		20:0,22:0,
24:0,hFA 		PubMed Felis
silvestris catus
/brain
(GM1,GD1a,GD1b,
GT1b,GQ1b) 		undescribed PubMed a-series,
b-series 		Canis lupus
familiaris
/brain 		undescribed PubMed
奇蹄目
Perissodactyla 		ウマ Equus
caballus
/brain
(Gal-Cer,SO3-3Galβ1-Cer) 		22:0,24:0,
24:1,h22:0,
h24:0,h24:1,
d18:1 		PubMed Equus
caballus
/spleen 		16:0,22:0,
24:0 		PubMed Equus
caballus
/brain
(GM4,GM3,GM2,
GM1,GD1a,GD1b,
GT1b) 		18:0,20:0,
24:0,h24:0,
h24:1,d18:1,
d20:1 		PubMed a-series,
b-series 		Equus
caballus
/spinal cord 		16:0,18:0,
22:0,24:1 		DOI
鯨偶蹄目
Cetartiodactyla 		ウシ、ブタ Bos
taurus
/brain
(Gal-Cer,SO3-3Galβ1-Cer) 		18:0,24:0,
24:1,h18:0,
h24:0,h24:1,
d18:1 		PubMed Bos
taurus
/spleen 		22:0,23:0,
24:0 		PubMed Bos
taurus
/brain
(GM2,GM1,GD3,
GD1a,GD1b) 		undescribed PubMed a-series,
b-series 		Bos
taurus
/brain 		18:0,24:0,24:1,
d18:1 		PubMed Ovis
aries
/brain,
Capra
linnaeus
/brain 		undescribed PubMed
兎目
Lagomorpha 		ウサギ Oryctolagus
cuniculus
/brain
(Gal-Cer,SO3-3Galβ1-Cer) 		undescribed PubMed Oryctolagus
cuniculus
/skin fibroblasts 		undescribed PubMed Oryctolagus
cuniculus
/brain
(GM1,GD1a,GD1b,
GT1) 		undescribed PubMed a-series,
b-series 		Oryctolagus
cuniculus
/brain 		undescribed PubMed
齧歯目
Rodentia 		ラット、マウス Rattus
norvegicus
/brain
(Gal-Cer,SO3-3Galβ1-Cer) 		undescribed PubMed Mus
musculus
/erythrocytes 		16:0,18:1,
22:0,24:1,
d18:1 		PubMed Rattus
norvegicus
/brain
(GM1,GD1a,GD1b,
GT) 		undescribed PubMed a-series,
b-series 		Rattus
norvegicus
/brain 		undescribed PubMed
霊長目
Primates 		ヒト Primates.png Homo
sapiens
/brain
(Gal-Cer,SO3-3Galβ1-Cer) 		18:0,24:1,
24:0,25:1,
25:0,26:1,
h16:0,h22:0,
h23:0,h24:1,
h24:0 		PubMed Homo
sapiens
/spleen of
Gaucher's
disease

Homo
sapiens
/blood 		22:0,d18:1

undescribed 		PubMed

PubMed 		Homo
sapiens
/brain
(GM1,GD1a,GD1b,
GT1b) 		undescribed PubMed a-series,
b-series 		Homo
sapiens
/brain 		18:0,24:1 PubMed

Biosynthesis

In Mammalia, GlcCer is synthesized by ceramide glucosyltransferase (CGlcT) at the cytosolic membrane of the Golgi apparatus, and GalCer is synthesized by ceramide galactosyltransferase (CGalT) at the lumen of the endoplasmic reticulum (ER). These enzymes do not share any sequence similarity and CGlcT possesses its transmembrane region at the N-terminal (type III) whereas CGalT possesses it at the C-terminal (type I).[1]

哺乳動物では、ゴルジ装置の細胞質側に局在するグルコシルセラミド合成酵素（CGlcT）によって GlcCer が、小胞体の内腔側に局在するガラクトシルセラミド合成酵素（CGalT）によって GalCer が作られます。これらの酵素は配列相同性が無く、CGlcT は膜貫通領域が N 末端側（III型酵素）、CGalT は C 末端側（I型酵素）です。

GlcCer GalCer
Enzyme CGlcT (EC 2.4.1.80) CGalT (EC 2.4.1.45)
Reaction Ceramide + UDP-glucose → GlcCer + UDP Ceramide + UDP-galactose → GalCer + UDP
Location Golgi (outside) → flipflop → Golgi (inside) ER lumen

Since all glycolipid synthesizing enzymes (except for CGlcT and CGalT) reside at the lumen of the Golgi apparatus, GlcCer must be flip-flopped from the cytosolic side to the lumen by an eyzyme called flippase. To synthesize GalCer, UDP-galactose and ceramide must transport the ER membrane. UDP-galactose transporter 2 (UGT2) is considered to be responsible.

GalT can also work on glycerolipid, and synthsizes sulfated glyceroglycolipid in mammalian testis, called seminolipid.

CGlcT, CGalT 以外の糖脂質合成酵素はゴルジ装置の内腔側にあり、GlcCer はゴルジ膜の細胞質側から内腔側にフリッパーゼと呼ばれる酵素で移動（フリップフロップ）します。GalCer の場合は、UDPガラクトースとセラミドが小胞体膜を通過します。UDPガラクトーストランスポータ2 (UGT2) が関与するようです。

CGalTはグリセロ脂質にも作用し、精巣の硫酸化グリセロ糖脂質であるセミノリピドも合成します。

  1. ↑ Sprong H, Degroote S, Nilsson T, Kawakita M, Ishida N, van der Sluijs P, van Meer G "Association of the Golgi UDP-galactose transporter with UDP-galactose:ceramide galactosyltransferase allows UDP-galactose import in the endoplasmic reticulum" Mol Biol Cell. 2003;4:3482-93　PMID 12925779

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