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Human Metabolome Database Version 3.5

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Showing metabocard for Ceramide (d18:1/25:0) (HMDB04957)

Record Information
Version 3.5
Creation Date 2005-11-16 08:48:42 -0700
Update Date 2013-02-08 17:13:40 -0700
HMDB ID HMDB04957
Secondary Accession Numbers None
Metabolite Identification
Common Name Ceramide (d18:1/25:0)
Description Ceramides (N-acylsphingosine) are one of the hydrolysis byproducts of sphingomyelin by the enzyme sphingomyelinase (sphingomyelin phosphorylcholine phosphohydrolase E.C.3.1.4.12) which has been identified in the subcellular fractions of human epidermis (PMID 25935 Link_out) and many other tissues. They can also be synthesized from serine and palmitate in a de novo pathway and are regarded as important cellular signals for inducing apoptosis (PMID 14998372 Link_out). Is key in the biosynthesis of glycosphingolipids and gangliosides.
Structure Thumb
Download: MOL | SDF | SMILES | InChI
Display: 2D Structure | 3D Structure
Synonyms
  1. (2S,3R,4E)-2-acylamino-1,3-octadec-4-enediol
  2. (2S,3R,4E)-2-acylaminooctadec-4-ene-1,3-diol
  3. Cer
  4. Ceramide
  5. N-Acylsphingosine
  6. N-[(1S,2R,3E)-2-Hydroxy-1-(hydroxymethyl)-3-heptadecenyl]-octadecanamide
Chemical Formula C43H85NO3
Average Molecular Weight 664.1399
Monoisotopic Molecular Weight 663.652945591
IUPAC Name N-[(2S,3R,4E)-1,3-dihydroxyoctadec-4-en-2-yl]pentacosanamide
Traditional IUPAC Name N-[(2S,3R,4E)-1,3-dihydroxyoctadec-4-en-2-yl]pentacosanamide
CAS Registry Number 104404-17-3
SMILES CCCCCCCCCCCCCCCCCCCCCCCCC(=O)N[C@@H](CO)[C@H](O)\C=C\CCCCCCCCCCCCC
InChI Identifier InChI=1S/C43H85NO3/c1-3-5-7-9-11-13-15-17-18-19-20-21-22-23-24-25-27-29-31-33-35-37-39-43(47)44-41(40-45)42(46)38-36-34-32-30-28-26-16-14-12-10-8-6-4-2/h36,38,41-42,45-46H,3-35,37,39-40H2,1-2H3,(H,44,47)/b38-36+/t41-,42+/m0/s1
InChI Key QHPYSHVSWAOLHS-PVNBSDFKSA-N
Chemical Taxonomy
Kingdom Organic Compounds
Super Class Lipids
Class Sphingolipids
Sub Class Ceramides
Other Descriptors
  • Aliphatic Acyclic Compounds
Substituents
  • Acyclic Alkene
  • Allyl Alcohol
  • Carboxamide Group
  • N Acyl Amine
  • Primary Alcohol
  • Secondary Alcohol
  • Secondary Carboxylic Acid Amide
Direct Parent Ceramides
Ontology
Status Detected and Quantified
Origin
  • Endogenous
  • Food
Biofunction
  • Cell signaling
  • Component of Glycerolipid metabolism
  • Component of Glycerophospholipid metabolism
  • Component of Glycosphingolipid metabolism
  • Component of Prostaglandin and leukotriene metabolism
  • Fuel and energy storage
  • Fuel or energy source
  • Membrane integrity/stability
  • Second messenger
Application
  • Nutrients
  • Stabilizers
  • Surfactants and Emulsifiers
Cellular locations
  • Extracellular
  • Membrane
Physical Properties
State Solid
Experimental Properties
Property Value Reference
Melting Point Not Available Not Available
Boiling Point Not Available Not Available
Water Solubility Insoluble Not Available
LogP Not Available Not Available
Predicted Properties
Property Value Source
Water Solubility 2.120E-05 g/L ALOGPS
LogP 10.39 ALOGPS
LogP 14.87 ChemAxon
LogS -7.50 ALOGPS
pKa (strongest acidic) 13.62 ChemAxon
pKa (strongest basic) 0.02 ChemAxon
Hydrogen Acceptor Count 3 ChemAxon
Hydrogen Donor Count 3 ChemAxon
Polar Surface Area 69.56 A2 ChemAxon
Rotatable Bond Count 39 ChemAxon
Refractivity 207.18 ChemAxon
Polarizability 92.47 ChemAxon
Formal Charge 0 ChemAxon
Physiological Charge 0 ChemAxon
Spectra
Not Available
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane
Biofluid Locations
  • Blood
Tissue Location
  • Muscle
  • Skeletal Muscle
  • Fibroblasts
  • Intestine
  • Neuron
  • Pancreas
  • Placenta
  • Testes
  • Kidney
  • Liver
  • Thyroid Gland
  • Brain
  • Myelin
  • Skin
  • Nerve Cells
  • Platelet
  • Spleen
  • Stratum Corneum
  • Keratinocyte
Pathways Not Available
Normal Concentrations
Biofluid Status Value Age Sex Condition Comments
Blood Detected and Quantified
Article_icon
0.022 +/- 0.001 uM Adult (>18 years old) Both Normal Not Available
Blood Detected and Quantified
Article_icon
0.128 +/- 0.012 uM Adult (>18 years old) Both Normal Not Available
Blood Detected and Quantified
Article_icon
0.040 +/- 0.002 uM Adult (>18 years old) Both Normal Not Available
Blood Detected and Quantified
Article_icon
0.145 +/- 0.007 uM Adult (>18 years old) Both Normal Not Available
Blood Detected and Quantified
Article_icon
1.22 +/- 0.046 uM Adult (>18 years old) Both Normal Not Available
Blood Detected and Quantified
Article_icon
0.281 +/- 0.033 uM Adult (>18 years old) Both Normal Not Available
Blood Detected and Quantified
Article_icon
1.00 +/- 0.029 uM Adult (>18 years old) Both Normal Not Available
Blood Detected and Quantified
Article_icon
3.00 +/- 0.107 uM Adult (>18 years old) Both Normal Not Available
Blood Detected and Quantified
Article_icon
0.271 +/- 0.013 uM Adult (>18 years old) Both Normal Not Available
Blood Detected and Quantified
Article_icon
0.036 +/- 0.002 uM Adult (>18 years old) Both Normal Not Available
Blood Detected and Quantified
Article_icon
0.061 +/- 0.0007 uM Adult (>18 years old) Both Normal Not Available
Blood Detected and Quantified
Article_icon
0.012 +/- 0.001 uM Adult (>18 years old) Both Normal Not Available
Blood Detected and Quantified
Article_icon
0.331 +/- 0.029 uM Adult (>18 years old) Both Normal Not Available
Blood Detected and Quantified
Article_icon
0.008 +/- 0.001 uM Adult (>18 years old) Both Normal Not Available
Abnormal Concentrations
Not Available
Associated Disorders and Diseases
Disease References None
Associated OMIM IDs None
DrugBank ID Not Available
Phenol Explorer Compound ID Not Available
Phenol Explorer Metabolite ID Not Available
FoodDB ID FDB023544
KNApSAcK ID Not Available
Chemspider ID 7826144 Link_out
KEGG Compound ID C00195 Link_out
BioCyc ID CERAMIDE Link_out
BiGG ID Not Available
Wikipedia Link Cer Link_out
NuGOwiki Link HMDB04957 Link_out
Metagene Link HMDB04957 Link_out
METLIN ID 7210 Link_out
PubChem Compound 9547202 Link_out
PDB ID Not Available
ChEBI ID Not Available
References
Synthesis Reference Not Available
Material Safety Data Sheet (MSDS) Not Available
General References
  1. Deguchi H, Yegneswaran S, Griffin JH: Sphingolipids as bioactive regulators of thrombin generation. J Biol Chem. 2004 Mar 26;279(13):12036-42. Epub 2004 Jan 13. Pubmed: 14722105 Link_out
  2. Watanabe R, Wu K, Paul P, Marks DL, Kobayashi T, Pittelkow MR, Pagano RE: Up-regulation of glucosylceramide synthase expression and activity during human keratinocyte differentiation. J Biol Chem. 1998 Apr 17;273(16):9651-5. Pubmed: 9545298 Link_out
  3. Mari M, Colell A, Morales A, Paneda C, Varela-Nieto I, Garcia-Ruiz C, Fernandez-Checa JC: Acidic sphingomyelinase downregulates the liver-specific methionine adenosyltransferase 1A, contributing to tumor necrosis factor-induced lethal hepatitis. J Clin Invest. 2004 Mar;113(6):895-904. Pubmed: 15067322 Link_out
  4. Adams JM 2nd, Pratipanawatr T, Berria R, Wang E, DeFronzo RA, Sullards MC, Mandarino LJ: Ceramide content is increased in skeletal muscle from obese insulin-resistant humans. Diabetes. 2004 Jan;53(1):25-31. Pubmed: 14693694 Link_out
  5. Holleran WM, Ginns EI, Menon GK, Grundmann JU, Fartasch M, McKinney CE, Elias PM, Sidransky E: Consequences of beta-glucocerebrosidase deficiency in epidermis. Ultrastructure and permeability barrier alterations in Gaucher disease. J Clin Invest. 1994 Apr;93(4):1756-64. Pubmed: 8163674 Link_out
  6. Marchesini S, Demasi L, Cestone P, Preti A, Agmon V, Dagan A, Navon R, Gatt S: Sulforhodamine GM1-ganglioside: synthesis and physicochemical properties. Chem Phys Lipids. 1994 Aug 8;72(2):143-52. Pubmed: 7954976 Link_out
  7. Poliak S, Gollan L, Salomon D, Berglund EO, Ohara R, Ranscht B, Peles E: Localization of Caspr2 in myelinated nerves depends on axon-glia interactions and the generation of barriers along the axon. J Neurosci. 2001 Oct 1;21(19):7568-75. Pubmed: 11567047 Link_out
  8. Ohnishi Y, Okino N, Ito M, Imayama S: Ceramidase activity in bacterial skin flora as a possible cause of ceramide deficiency in atopic dermatitis. Clin Diagn Lab Immunol. 1999 Jan;6(1):101-4. Pubmed: 9874672 Link_out
  9. Humbert P: [Functional consequences of cutaneous lipid perturbation] Pathol Biol (Paris). 2003 Jul;51(5):271-4. Pubmed: 14567193 Link_out
  10. Ghadially R, Brown BE, Sequeira-Martin SM, Feingold KR, Elias PM: The aged epidermal permeability barrier. Structural, functional, and lipid biochemical abnormalities in humans and a senescent murine model. J Clin Invest. 1995 May;95(5):2281-90. Pubmed: 7738193 Link_out
  11. Ogawa-Goto K, Funamoto N, Abe T, Nagashima K: Different ceramide compositions of gangliosides between human motor and sensory nerves. J Neurochem. 1990 Nov;55(5):1486-93. Pubmed: 2213006 Link_out
  12. Cho Y, Lew BL, Seong K, Kim NI: An inverse relationship between ceramide synthesis and clinical severity in patients with psoriasis. J Korean Med Sci. 2004 Dec;19(6):859-63. Pubmed: 15608398 Link_out
  13. Bouwstra JA, Honeywell-Nguyen PL, Gooris GS, Ponec M: Structure of the skin barrier and its modulation by vesicular formulations. Prog Lipid Res. 2003 Jan;42(1):1-36. Pubmed: 12467638 Link_out
  14. Uchida Y, Behne M, Quiec D, Elias PM, Holleran WM: Vitamin C stimulates sphingolipid production and markers of barrier formation in submerged human keratinocyte cultures. J Invest Dermatol. 2001 Nov;117(5):1307-13. Pubmed: 11710949 Link_out
  15. Petrache I, Natarajan V, Zhen L, Medler TR, Richter AT, Cho C, Hubbard WC, Berdyshev EV, Tuder RM: Ceramide upregulation causes pulmonary cell apoptosis and emphysema-like disease in mice. Nat Med. 2005 May;11(5):491-8. Epub 2005 Apr 24. Pubmed: 15852018 Link_out
  16. Demarchi F, Bertoli C, Greer PA, Schneider C: Ceramide triggers an NF-kappaB-dependent survival pathway through calpain. Cell Death Differ. 2005 May;12(5):512-22. Pubmed: 15933726 Link_out
  17. Schafer A, Harzer K, Kattner E, Schafer HJ, Stoltenburg G, Lietz H: [Disseminated lipogranulomatosis (Farber disease) with hydrops fetalis] Pathologe. 1996 Mar;17(2):145-9. Pubmed: 8650144 Link_out
  18. Pettus BJ, Baes M, Busman M, Hannun YA, Van Veldhoven PP: Mass spectrometric analysis of ceramide perturbations in brain and fibroblasts of mice and human patients with peroxisomal disorders. Rapid Commun Mass Spectrom. 2004;18(14):1569-74. Pubmed: 15282781 Link_out
  19. Okamoto R, Arikawa J, Ishibashi M, Kawashima M, Takagi Y, Imokawa G: Sphingosylphosphorylcholine is upregulated in the stratum corneum of patients with atopic dermatitis. J Lipid Res. 2003 Jan;44(1):93-102. Pubmed: 12518027 Link_out
  20. Lew BL, Cho Y, Kim J, Sim WY, Kim NI: Ceramides and cell signaling molecules in psoriatic epidermis: reduced levels of ceramides, PKC-alpha, and JNK. J Korean Med Sci. 2006 Feb;21(1):95-9. Pubmed: 16479073 Link_out
  21. van Lijnschoten G, Groener JE, Maas SM, Ben-Yoseph Y, Dingemans KP, Offerhaus GJ: Intrauterine fetal death due to Farber disease: case report. Pediatr Dev Pathol. 2000 Nov-Dec;3(6):597-602. Pubmed: 11000338 Link_out
  22. Jana A, Pahan K: Human immunodeficiency virus type 1 gp120 induces apoptosis in human primary neurons through redox-regulated activation of neutral sphingomyelinase. J Neurosci. 2004 Oct 27;24(43):9531-40. Pubmed: 15509740 Link_out
  23. Klein J: Functions and pathophysiological roles of phospholipase D in the brain. J Neurochem. 2005 Sep;94(6):1473-87. Epub 2005 Jul 22. Pubmed: 16042758 Link_out
  24. Yatomi Y, Yamamura S, Hisano N, Nakahara K, Igarashi Y, Ozaki Y: Sphingosine 1-phosphate breakdown in platelets. J Biochem (Tokyo). 2004 Oct;136(4):495-502. Pubmed: 15625319 Link_out
  25. Grether-Beck S, Bonizzi G, Schmitt-Brenden H, Felsner I, Timmer A, Sies H, Johnson JP, Piette J, Krutmann J: Non-enzymatic triggering of the ceramide signalling cascade by solar UVA radiation. EMBO J. 2000 Nov 1;19(21):5793-800. Pubmed: 11060030 Link_out
  26. Ogawa-Goto K, Ohta Y, Kubota K, Funamoto N, Abe T, Taki T, Nagashima K: Glycosphingolipids of human peripheral nervous system myelins isolated from cauda equina. J Neurochem. 1993 Oct;61(4):1398-403. Pubmed: 7690848 Link_out
  27. Satoi H, Tomimoto H, Ohtani R, Kitano T, Kondo T, Watanabe M, Oka N, Akiguchi I, Furuya S, Hirabayashi Y, Okazaki T: Astroglial expression of ceramide in Alzheimer's disease brains: a role during neuronal apoptosis. Neuroscience. 2005;130(3):657-66. Pubmed: 15590150 Link_out
  28. Garcia-Ruiz C, Mari M, Morales A, Colell A, Ardite E, Fernandez-Checa JC: Human placenta sphingomyelinase, an exogenous acidic pH-optimum sphingomyelinase, induces oxidative stress, glutathione depletion, and apoptosis in rat hepatocytes. Hepatology. 2000 Jul;32(1):56-65. Pubmed: 10869289 Link_out
  29. Gill JS, Windebank AJ: Suramin induced ceramide accumulation leads to apoptotic cell death in dorsal root ganglion neurons. Cell Death Differ. 1998 Oct;5(10):876-83. Pubmed: 10203686 Link_out
  30. Both DM, Goodtzova K, Yarosh DB, Brown DA: Liposome-encapsulated ursolic acid increases ceramides and collagen in human skin cells. Arch Dermatol Res. 2002 Jan;293(11):569-75. Pubmed: 11876525 Link_out
  31. Yarosh DB, Both D, Brown D: Liposomal ursolic acid (merotaine) increases ceramides and collagen in human skin. Horm Res. 2000;54(5-6):318-21. Pubmed: 11595826 Link_out
  32. Dunn HG, Dolman CL, Farrell DF, Tischler B, Hasinoff C, Woolf LI: Krabbe's leukodystrophy without globoid cells. Neurology. 1976 Nov;26(11):1035-41. Pubmed: 988509 Link_out
  33. Persaud-Sawin DA, Boustany RM: Cell death pathways in juvenile Batten disease. Apoptosis. 2005 Oct;10(5):973-85. Pubmed: 16151633 Link_out
  34. Di Marzio L, Cinque B, De Simone C, Cifone MG: Effect of the lactic acid bacterium Streptococcus thermophilus on ceramide levels in human keratinocytes in vitro and stratum corneum in vivo. J Invest Dermatol. 1999 Jul;113(1):98-106. Pubmed: 10417626 Link_out
  35. Kirby RJ, Zheng S, Tso P, Howles PN, Hui DY: Bile salt-stimulated carboxyl ester lipase influences lipoprotein assembly and secretion in intestine: a process mediated via ceramide hydrolysis. J Biol Chem. 2002 Feb 8;277(6):4104-9. Epub 2001 Dec 3. Pubmed: 11733511 Link_out
  36. Tanno O, Ota Y, Kitamura N, Katsube T, Inoue S: Nicotinamide increases biosynthesis of ceramides as well as other stratum corneum lipids to improve the epidermal permeability barrier. Br J Dermatol. 2000 Sep;143(3):524-31. Pubmed: 10971324 Link_out
  37. Malagarie-Cazenave S, Segui B, Leveque S, Garcia V, Carpentier S, Altie MF, Brouchet A, Gouaze V, Andrieu-Abadie N, Barreira Y, Benoist H, Levade T: Role of FAN in tumor necrosis factor-alpha and lipopolysaccharide-induced interleukin-6 secretion and lethality in D-galactosamine-sensitized mice. J Biol Chem. 2004 Apr 30;279(18):18648-55. Epub 2004 Feb 25. Pubmed: 14985352 Link_out
  38. Farina F, Cappello F, Todaro M, Bucchieri F, Peri G, Zummo G, Stassi G: Involvement of caspase-3 and GD3 ganglioside in ceramide-induced apoptosis in Farber disease. J Histochem Cytochem. 2000 Jan;48(1):57-62. Pubmed: 10653586 Link_out
  39. Sugiki H, Hozumi Y, Maeshima H, Katagata Y, Mitsuhashi Y, Kondo S: C2-ceramide induces apoptosis in a human squamous cell carcinoma cell line. Br J Dermatol. 2000 Dec;143(6):1154-63. Pubmed: 11122015 Link_out
  40. Chavez JA, Holland WL, Bar J, Sandhoff K, Summers SA: Acid ceramidase overexpression prevents the inhibitory effects of saturated fatty acids on insulin signaling. J Biol Chem. 2005 May 20;280(20):20148-53. Epub 2005 Mar 17. Pubmed: 15774472 Link_out
  41. Saito M, Saito M, Cooper TB, Vadasz C: Ethanol-induced changes in the content of triglycerides, ceramides, and glucosylceramides in cultured neurons. Alcohol Clin Exp Res. 2005 Aug;29(8):1374-83. Pubmed: 16131844 Link_out
  42. Maurer BJ, Melton L, Billups C, Cabot MC, Reynolds CP: Synergistic cytotoxicity in solid tumor cell lines between N-(4-hydroxyphenyl)retinamide and modulators of ceramide metabolism. J Natl Cancer Inst. 2000 Dec 6;92(23):1897-909. Pubmed: 11106681 Link_out
  43. Lee HK, Nam GW, Kim SH, Lee SH: Phytocomponents of triterpenoids, oleanolic acid and ursolic acid, regulated differently the processing of epidermal keratinocytes via PPAR-alpha pathway. Exp Dermatol. 2006 Jan;15(1):66-73. Pubmed: 16364033 Link_out
  44. Di Marzio L, Centi C, Cinque B, Masci S, Giuliani M, Arcieri A, Zicari L, De Simone C, Cifone MG: Effect of the lactic acid bacterium Streptococcus thermophilus on stratum corneum ceramide levels and signs and symptoms of atopic dermatitis patients. Exp Dermatol. 2003 Oct;12(5):615-20. Pubmed: 14705802 Link_out
  45. Sando GN, Howard EJ, Madison KC: Induction of ceramide glucosyltransferase activity in cultured human keratinocytes. Correlation with culture differentiation. J Biol Chem. 1996 Sep 6;271(36):22044-51. Pubmed: 8703011 Link_out
  46. Helge JW, Dobrzyn A, Saltin B, Gorski J: Exercise and training effects on ceramide metabolism in human skeletal muscle. Exp Physiol. 2004 Jan;89(1):119-27. Pubmed: 15109217 Link_out
  47. Bektas M, Orfanos CE, Geilen CC: Different vitamin D analogues induce sphingomyelin hydrolysis and apoptosis in the human keratinocyte cell line HaCaT. Cell Mol Biol (Noisy-le-grand). 2000 Feb;46(1):111-9. Pubmed: 10726977 Link_out
  48. Guchhait P, Lopez JA, Thiagarajan P: Characterization of autoantibodies against sulfatide from a V-gene phage-display library derived from patients with systemic lupus erythematosus. J Immunol Methods. 2004 Dec;295(1-2):129-37. Epub 2004 Oct 26. Pubmed: 15627618 Link_out
  49. Erdreich-Epstein A, Tran LB, Cox OT, Huang EY, Laug WE, Shimada H, Millard M: Endothelial apoptosis induced by inhibition of integrins alphavbeta3 and alphavbeta5 involves ceramide metabolic pathways. Blood. 2005 Jun 1;105(11):4353-61. Epub 2005 Feb 10. Pubmed: 15705795 Link_out
  50. Herr I, Martin-Villalba A, Kurz E, Roncaioli P, Schenkel J, Cifone MG, Debatin KM: FK506 prevents stroke-induced generation of ceramide and apoptosis signaling. Brain Res. 1999 May 1;826(2):210-9. Pubmed: 10224298 Link_out
  51. Futerman AH: The roles of ceramide in the regulation of neuronal growth and development. Biochemistry (Mosc). 1998 Jan;63(1):74-83. Pubmed: 9526098 Link_out
  52. Vielhaber G, Pfeiffer S, Brade L, Lindner B, Goldmann T, Vollmer E, Hintze U, Wittern KP, Wepf R: Localization of ceramide and glucosylceramide in human epidermis by immunogold electron microscopy. J Invest Dermatol. 2001 Nov;117(5):1126-36. Pubmed: 11710923 Link_out
  53. Tojo K, Oota M, Honda H, Shibasaki T, Sakai O: Possible thyroidal involvement in a case of Fabry disease. Intern Med. 1994 Mar;33(3):172-6. Pubmed: 8061397 Link_out

Enzymes
Name: Lipid phosphate phosphohydrolase 2
Reactions:
  • a 1,2-diacylglycerol 3-phosphate + H2O = a 1,2-diacyl-sn-glycerol + phosphate [RN:R02239]
Gene Name: PPAP2C
Uniprot ID: O43688 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Lipid phosphate phosphohydrolase 1
Reactions:
  • a 1,2-diacylglycerol 3-phosphate + H2O = a 1,2-diacyl-sn-glycerol + phosphate [RN:R02239]
Gene Name: PPAP2A
Uniprot ID: O14494 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Lipid phosphate phosphohydrolase 3
Reactions:
  • a 1,2-diacylglycerol 3-phosphate + H2O = a 1,2-diacyl-sn-glycerol + phosphate [RN:R02239]
Gene Name: PPAP2B
Uniprot ID: O14495 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Galactocerebrosidase
Reactions:
  • D-galactosyl-N-acylsphingosine + H2O = D-galactose + N-acylsphingosine [RN:R03617]
Gene Name: GALC
Uniprot ID: P54803 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Lactase-phlorizin hydrolase
Reactions:
  • lactose + H2O = D-galactose + D-glucose [RN:R01100 R06114]
Gene Name: LCT
Uniprot ID: P09848 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Globoside alpha-1,3-N-acetylgalactosaminyltransferase 1
Reactions:
    Gene Name: GBGT1
    Uniprot ID: Q8N5D6 Link_out
    Protein Sequence: FASTA
    Gene Sequence: FASTA
    Name: N-acetylglucosaminyl-phosphatidylinositol de-N-acetylase
    Reactions:
    • 6-(N-acetyl-alpha-D-glucosaminyl)-1-phosphatidyl-1D-myo-inositol + H2O = 6-(alpha-D-glucosaminyl)-1-phosphatidyl-1D-myo-inositol + acetate [RN:R03482 R05917]
    Gene Name: PIGL
    Uniprot ID: Q9Y2B2 Link_out
    Protein Sequence: FASTA
    Gene Sequence: FASTA
    Name: Phosphatidylcholine:ceramide cholinephosphotransferase 2
    Reactions:
    • a ceramide + a phosphatidylcholine = a sphingomyelin + a 1,2-diacyl-sn-glycerol [RN:R08969]
    Gene Name: SGMS2
    Uniprot ID: Q8NHU3 Link_out
    Protein Sequence: FASTA
    Gene Sequence: FASTA
    Name: Phosphatidylcholine:ceramide cholinephosphotransferase 1
    Reactions:
    • a ceramide + a phosphatidylcholine = a sphingomyelin + a 1,2-diacyl-sn-glycerol [RN:R08969]
    Gene Name: SGMS1
    Uniprot ID: Q86VZ5 Link_out
    Protein Sequence: FASTA
    Gene Sequence: FASTA
    Name: Sphingomyelin phosphodiesterase
    Reactions:
    • sphingomyelin + H2O = N-acylsphingosine + choline phosphate [RN:R02541]
    Gene Name: SMPD1
    Uniprot ID: P17405 Link_out
    Protein Sequence: FASTA
    Gene Sequence: FASTA
    Name: Ceramide glucosyltransferase
    Reactions:
    • UDP-glucose + an N-acylsphingosine = UDP + a D-glucosyl-N-acylsphingosine [RN:R01497 R06275]
    Gene Name: UGCG
    Uniprot ID: Q16739 Link_out
    Protein Sequence: FASTA
    Gene Sequence: FASTA
    Name: Phosphatidylinositol N-acetylglucosaminyltransferase subunit Q
    Reactions:
    • UDP-N-acetyl-D-glucosamine + 1-phosphatidyl-1D-myo-inositol = UDP + 6-(N-acetyl-alpha-D-glucosaminyl)-1-phosphatidyl-1D-myo-inositol [RN:R02654 R05916]
    Gene Name: PIGQ
    Uniprot ID: Q9BRB3 Link_out
    Protein Sequence: FASTA
    Gene Sequence: FASTA
    Name: Phosphatidylinositol N-acetylglucosaminyltransferase subunit A
    Reactions:
    • UDP-N-acetyl-D-glucosamine + 1-phosphatidyl-1D-myo-inositol = UDP + 6-(N-acetyl-alpha-D-glucosaminyl)-1-phosphatidyl-1D-myo-inositol [RN:R02654 R05916]
    Gene Name: PIGA
    Uniprot ID: P37287 Link_out
    Protein Sequence: FASTA
    Gene Sequence: FASTA
    Name: Phosphatidylinositol N-acetylglucosaminyltransferase subunit H
    Reactions:
    • UDP-N-acetyl-D-glucosamine + 1-phosphatidyl-1D-myo-inositol = UDP + 6-(N-acetyl-alpha-D-glucosaminyl)-1-phosphatidyl-1D-myo-inositol [RN:R02654 R05916]
    Gene Name: PIGH
    Uniprot ID: Q14442 Link_out
    Protein Sequence: FASTA
    Gene Sequence: FASTA
    Name: Phosphatidylinositol N-acetylglucosaminyltransferase subunit P
    Reactions:
    • UDP-N-acetyl-D-glucosamine + 1-phosphatidyl-1D-myo-inositol = UDP + 6-(N-acetyl-alpha-D-glucosaminyl)-1-phosphatidyl-1D-myo-inositol [RN:R02654 R05916]
    Gene Name: PIGP
    Uniprot ID: P57054 Link_out
    Protein Sequence: FASTA
    Gene Sequence: FASTA
    Name: Phosphatidylinositol N-acetylglucosaminyltransferase subunit C
    Reactions:
    • UDP-N-acetyl-D-glucosamine + 1-phosphatidyl-1D-myo-inositol = UDP + 6-(N-acetyl-alpha-D-glucosaminyl)-1-phosphatidyl-1D-myo-inositol [RN:R02654 R05916]
    Gene Name: PIGC
    Uniprot ID: Q92535 Link_out
    Protein Sequence: FASTA
    Gene Sequence: FASTA
    Name: Sphingosine-1-phosphate lyase 1
    Reactions:
    • sphinganine 1-phosphate = phosphoethanolamine + palmitaldehyde [RN:R02464]
    Gene Name: SGPL1
    Uniprot ID: O95470 Link_out
    Protein Sequence: FASTA
    Gene Sequence: FASTA
    Name: Sphingosine kinase 1
    Reactions:
    • ATP + sphinganine = ADP + sphinganine 1-phosphate [RN:R02976]
    Gene Name: SPHK1
    Uniprot ID: Q9NYA1 Link_out
    Protein Sequence: FASTA
    Gene Sequence: FASTA
    Name: Acid ceramidase
    Reactions:
    • N-acylsphingosine + H2O = a carboxylate + sphingosine [RN:R01493]
    Gene Name: ASAH1
    Uniprot ID: Q13510 Link_out
    Protein Sequence: FASTA
    Gene Sequence: FASTA
    Name: Ectonucleotide pyrophosphatase/phosphodiesterase family member 7
    Reactions:
    • sphingomyelin + H2O = N-acylsphingosine + choline phosphate [RN:R02541]
    Gene Name: ENPP7
    Uniprot ID: Q6UWV6 Link_out
    Protein Sequence: FASTA
    Gene Sequence: FASTA
    Name: Sphingomyelin phosphodiesterase 2
    Reactions:
    • sphingomyelin + H2O = N-acylsphingosine + choline phosphate [RN:R02541]
    Gene Name: SMPD2
    Uniprot ID: O60906 Link_out
    Protein Sequence: FASTA
    Gene Sequence: FASTA
    Name: 2-hydroxyacylsphingosine 1-beta-galactosyltransferase
    Reactions:
    • UDP-galactose + 2-(2-hydroxyacyl)sphingosine = UDP + 1-(beta-D-galactosyl)-2-(2-hydroxyacyl)sphingosine [RN:R04322]
    Gene Name: UGT8
    Uniprot ID: Q16880 Link_out
    Protein Sequence: FASTA
    Gene Sequence: FASTA
    Name: Collagen type IV alpha-3-binding protein
    Reactions:
      Gene Name: COL4A3BP
      Uniprot ID: Q9Y5P4 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: Ceramide kinase
      Reactions:
      • ATP + ceramide = ADP + ceramide 1-phosphate [RN:R01495]
      Gene Name: CERK
      Uniprot ID: Q8TCT0 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: Ganglioside GM2 activator
      Reactions:
        Gene Name: GM2A
        Uniprot ID: P17900 Link_out
        Protein Sequence: FASTA
        Gene Sequence: FASTA
        Name: Glucosylceramidase
        Reactions:
        • D-glucosyl-N-acylsphingosine + H2O = D-glucose + N-acylsphingosine [RN:R01498]
        Gene Name: GBA
        Uniprot ID: P04062 Link_out
        Protein Sequence: FASTA
        Gene Sequence: FASTA
        Name: T-cell surface glycoprotein CD1e, membrane-associated
        Reactions:
          Gene Name: CD1E
          Uniprot ID: P15812 Link_out
          Protein Sequence: FASTA
          Gene Sequence: FASTA
          Name: Epididymal secretory protein E1
          Reactions:
            Gene Name: NPC2
            Uniprot ID: P61916 Link_out
            Protein Sequence: FASTA
            Gene Sequence: FASTA
            Name: LAG1 longevity assurance homolog 1
            Reactions:
              Gene Name: LASS1
              Uniprot ID: P27544 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Antigen-presenting glycoprotein CD1d
              Reactions:
                Gene Name: CD1D
                Uniprot ID: P15813 Link_out
                Protein Sequence: FASTA
                Gene Sequence: FASTA
                Name: GPI mannosyltransferase 1
                Reactions:
                  Gene Name: PIGM
                  Uniprot ID: Q9H3S5 Link_out
                  Protein Sequence: FASTA
                  Gene Sequence: FASTA
                  Name: Phosphatidylinositol-glycan biosynthesis class W protein
                  Reactions:
                    Gene Name: PIGW
                    Uniprot ID: Q7Z7B1 Link_out
                    Protein Sequence: FASTA
                    Gene Sequence: FASTA
                    Name: Phosphatidylinositol-glycan biosynthesis class X protein
                    Reactions:
                      Gene Name: PIGX
                      Uniprot ID: Q8TBF5 Link_out
                      Protein Sequence: FASTA
                      Gene Sequence: FASTA
                      Name: GPI mannosyltransferase 4
                      Reactions:
                        Gene Name: PIGZ
                        Uniprot ID: Q86VD9 Link_out
                        Protein Sequence: FASTA
                        Gene Sequence: FASTA
                        Name: UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 5
                        Reactions:
                          Gene Name: B3GNT5
                          Uniprot ID: Q9BYG0 Link_out
                          Protein Sequence: FASTA
                          Gene Sequence: FASTA
                          Name: Sphingomyelin phosphodiesterase 3
                          Reactions:
                          • sphingomyelin + H2O = N-acylsphingosine + choline phosphate [RN:R02541]
                          Gene Name: SMPD3
                          Uniprot ID: Q9NY59 Link_out
                          Protein Sequence: FASTA
                          Gene Sequence: FASTA
                          Name: Beta-1,3-galactosyltransferase 5
                          Reactions:
                            Gene Name: B3GALT5
                            Uniprot ID: Q9Y2C3 Link_out
                            Protein Sequence: FASTA
                            Gene Sequence: FASTA
                            Name: GPI mannosyltransferase 3
                            Reactions:
                              Gene Name: PIGB
                              Uniprot ID: Q92521 Link_out
                              Protein Sequence: FASTA
                              Gene Sequence: FASTA
                              Name: Phosphatidylinositol-glycan biosynthesis class F protein
                              Reactions:
                                Gene Name: PIGF
                                Uniprot ID: Q07326 Link_out
                                Protein Sequence: FASTA
                                Gene Sequence: FASTA
                                Name: GPI ethanolamine phosphate transferase 2
                                Reactions:
                                  Gene Name: PIGG
                                  Uniprot ID: Q5H8A4 Link_out
                                  Protein Sequence: FASTA
                                  Gene Sequence: FASTA
                                  Name: GPI ethanolamine phosphate transferase 1
                                  Reactions:
                                    Gene Name: PIGN
                                    Uniprot ID: O95427 Link_out
                                    Protein Sequence: FASTA
                                    Gene Sequence: FASTA
                                    Name: GPI ethanolamine phosphate transferase 3
                                    Reactions:
                                      Gene Name: PIGO
                                      Uniprot ID: Q8TEQ8 Link_out
                                      Protein Sequence: FASTA
                                      Gene Sequence: FASTA
                                      Name: GPI transamidase component PIG-S
                                      Reactions:
                                        Gene Name: PIGS
                                        Uniprot ID: Q96S52 Link_out
                                        Protein Sequence: FASTA
                                        Gene Sequence: FASTA
                                        Name: GPI transamidase component PIG-T
                                        Reactions:
                                          Gene Name: PIGT
                                          Uniprot ID: Q969N2 Link_out
                                          Protein Sequence: FASTA
                                          Gene Sequence: FASTA
                                          Name: Phosphatidylinositol glycan anchor biosynthesis class U protein
                                          Reactions:
                                            Gene Name: PIGU
                                            Uniprot ID: Q9H490 Link_out
                                            Protein Sequence: FASTA
                                            Gene Sequence: FASTA
                                            Name: GPI mannosyltransferase 2
                                            Reactions:
                                              Gene Name: PIGV
                                              Uniprot ID: Q9NUD9 Link_out
                                              Protein Sequence: FASTA
                                              Gene Sequence: FASTA
                                              Name: Phosphatidylinositol N-acetylglucosaminyltransferase subunit Y
                                              Reactions:
                                                Gene Name: PIGY
                                                Uniprot ID: Q3MUY2 Link_out
                                                Protein Sequence: FASTA
                                                Gene Sequence: FASTA
                                                Name: Pleckstrin homology domain-containing family A member 8
                                                Reactions:
                                                  Gene Name: PLEKHA8
                                                  Uniprot ID: Q96JA3 Link_out
                                                  Protein Sequence: FASTA
                                                  Gene Sequence: FASTA
                                                  Name: Sphingomyelin phosphodiesterase 4
                                                  Reactions:
                                                  • sphingomyelin + H2O = N-acylsphingosine + choline phosphate [RN:R02541]
                                                  Gene Name: SMPD4
                                                  Uniprot ID: Q9NXE4 Link_out
                                                  Protein Sequence: FASTA
                                                  Gene Sequence: FASTA
                                                  Name: Putative neutral ceramidase C
                                                  Reactions:
                                                  • N-acylsphingosine + H2O = a carboxylate + sphingosine [RN:R01493]
                                                  Gene Name: ASAH2C
                                                  Uniprot ID: P0C7U2 Link_out
                                                  Protein Sequence: FASTA
                                                  Gene Sequence: FASTA
                                                  Name: Alkaline ceramidase 2
                                                  Reactions:
                                                  • N-acylsphingosine + H2O = a carboxylate + sphingosine [RN:R01493]
                                                  Gene Name: ACER2
                                                  Uniprot ID: Q5QJU3 Link_out
                                                  Protein Sequence: FASTA
                                                  Gene Sequence: FASTA
                                                  Name: Neutral ceramidase
                                                  Reactions:
                                                  • N-acylsphingosine + H2O = a carboxylate + sphingosine [RN:R01493]
                                                  Gene Name: ASAH2
                                                  Uniprot ID: Q9NR71 Link_out
                                                  Protein Sequence: FASTA
                                                  Gene Sequence: FASTA
                                                  Name: Non-lysosomal glucosylceramidase
                                                  Reactions:
                                                  • D-glucosyl-N-acylsphingosine + H2O = D-glucose + N-acylsphingosine [RN:R01498]
                                                  Gene Name: GBA2
                                                  Uniprot ID: Q9HCG7 Link_out
                                                  Protein Sequence: FASTA
                                                  Gene Sequence: FASTA
                                                  Name: Alkaline ceramidase 1
                                                  Reactions:
                                                  • N-acylsphingosine + H2O = a carboxylate + sphingosine [RN:R01493]
                                                  Gene Name: ACER1
                                                  Uniprot ID: Q8TDN7 Link_out
                                                  Protein Sequence: FASTA
                                                  Gene Sequence: FASTA
                                                  Name: Killer cell lectin-like receptor subfamily B member 1
                                                  Reactions:
                                                    Gene Name: KLRB1
                                                    Uniprot ID: Q12918 Link_out
                                                    Protein Sequence: FASTA
                                                    Gene Sequence: FASTA
                                                    Name: Protein FAN
                                                    Reactions:
                                                      Gene Name: NSMAF
                                                      Uniprot ID: Q92636 Link_out
                                                      Protein Sequence: FASTA
                                                      Gene Sequence: FASTA
                                                      Name: LAG1 longevity assurance homolog 5
                                                      Reactions:
                                                        Gene Name: LASS5
                                                        Uniprot ID: Q8N5B7 Link_out
                                                        Protein Sequence: FASTA
                                                        Gene Sequence: FASTA
                                                        Name: ATP-binding cassette sub-family A member 7
                                                        Reactions:
                                                          Gene Name: ABCA7
                                                          Uniprot ID: Q8IZY2 Link_out
                                                          Protein Sequence: FASTA
                                                          Gene Sequence: FASTA
                                                          Name: GPI-anchor transamidase
                                                          Reactions:
                                                            Gene Name: PIGK
                                                            Uniprot ID: Q92643 Link_out
                                                            Protein Sequence: FASTA
                                                            Gene Sequence: FASTA
                                                            Name: Glycosylphosphatidylinositol anchor attachment 1 protein
                                                            Reactions:
                                                              Gene Name: GPAA1
                                                              Uniprot ID: O43292 Link_out
                                                              Protein Sequence: FASTA
                                                              Gene Sequence: FASTA
                                                              Name: Protein PLEKHA9
                                                              Reactions:
                                                                Gene Name: PLEKHA9
                                                                Uniprot ID: O95397 Link_out
                                                                Protein Sequence: FASTA
                                                                Gene Sequence: FASTA
                                                                Name: Glycolipid transfer protein domain-containing protein 2
                                                                Reactions:
                                                                  Gene Name: GLTPD2
                                                                  Uniprot ID: A6NH11 Link_out
                                                                  Protein Sequence: FASTA
                                                                  Gene Sequence: FASTA
                                                                  Name: Putative uncharacterized protein PLEKHA8
                                                                  Reactions:
                                                                    Gene Name: PLEKHA8
                                                                    Uniprot ID: B5MDU3 Link_out
                                                                    Protein Sequence: FASTA
                                                                    Gene Sequence: FASTA
                                                                    Name: T-cell surface glycoprotein CD1a
                                                                    Reactions:
                                                                      Gene Name: CD1A
                                                                      Uniprot ID: P06126 Link_out
                                                                      Protein Sequence: FASTA
                                                                      Gene Sequence: FASTA
                                                                      Name: Glycolipid transfer protein domain-containing protein 1
                                                                      Reactions:
                                                                        Gene Name: GLTPD1
                                                                        Uniprot ID: Q5TA50 Link_out
                                                                        Protein Sequence: FASTA
                                                                        Gene Sequence: FASTA
                                                                        Name: T-cell surface glycoprotein CD1c
                                                                        Reactions:
                                                                          Gene Name: CD1C
                                                                          Uniprot ID: P29017 Link_out
                                                                          Protein Sequence: FASTA
                                                                          Gene Sequence: FASTA
                                                                          Name: Lipopolysaccharide-binding protein
                                                                          Reactions:
                                                                            Gene Name: LBP
                                                                            Uniprot ID: P18428 Link_out
                                                                            Protein Sequence: FASTA
                                                                            Gene Sequence: FASTA
                                                                            Name: Putative uncharacterized protein DKFZp434L0435
                                                                            Reactions:
                                                                              Gene Name: DKFZp434L0435
                                                                              Uniprot ID: Q9UFH6 Link_out
                                                                              Protein Sequence: FASTA
                                                                              Gene Sequence: FASTA
                                                                              Name: T-cell surface glycoprotein CD1b
                                                                              Reactions:
                                                                                Gene Name: CD1B
                                                                                Uniprot ID: P29016 Link_out
                                                                                Protein Sequence: FASTA
                                                                                Gene Sequence: FASTA
                                                                                Name: Glycolipid transfer protein
                                                                                Reactions:
                                                                                  Gene Name: GLTP
                                                                                  Uniprot ID: Q9NZD2 Link_out
                                                                                  Protein Sequence: FASTA
                                                                                  Gene Sequence: FASTA