Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2013-05-29 19:41:17 UTC
HMDB IDHMDB04952
Secondary Accession NumbersNone
Metabolite Identification
Common NameCeramide (d18:1/22:0)
DescriptionCeramides (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 ) 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 ). Is key in the biosynthesis of glycosphingolipids and gangliosides.
Structure
Thumb
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 FormulaC40H79NO3
Average Molecular Weight622.0602
Monoisotopic Molecular Weight621.605995399
IUPAC NameN-[(2S,3R,4E)-1,3-dihydroxyoctadec-4-en-2-yl]docosanamide
Traditional IUPAC NameC22 cer
CAS Registry Number104404-17-3
SMILES
CCCCCCCCCCCCCCCCCCCCCC(=O)N[C@@H](CO)[C@H](O)\C=C\CCCCCCCCCCCCC
InChI Identifier
InChI=1S/C40H79NO3/c1-3-5-7-9-11-13-15-17-18-19-20-21-22-24-26-28-30-32-34-36-40(44)41-38(37-42)39(43)35-33-31-29-27-25-23-16-14-12-10-8-6-4-2/h33,35,38-39,42-43H,3-32,34,36-37H2,1-2H3,(H,41,44)/b35-33+/t38-,39+/m0/s1
InChI KeyKEPQASGDXIEOIL-GLQCRSEXSA-N
Chemical Taxonomy
KingdomOrganic Compounds
Super ClassLipids
ClassSphingolipids
Sub ClassCeramides
Other Descriptors
  • Aliphatic Acyclic Compounds
Substituents
  • Acyclic Alkene
  • Allyl Alcohol
  • Carboxamide Group
  • N Acyl Amine
  • Primary Alcohol
  • Secondary Alcohol
  • Secondary Carboxylic Acid Amide
Direct ParentCeramides
Ontology
StatusDetected 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
StateSolid
Experimental Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityInsolubleNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
water solubility2.510E-05 g/LALOGPS
logP10.21ALOGPS
logP13.53ChemAxon
logS-7.4ALOGPS
pKa (strongest acidic)13.62ChemAxon
pKa (strongest basic)0.02ChemAxon
physiological charge0ChemAxon
hydrogen acceptor count3ChemAxon
hydrogen donor count3ChemAxon
polar surface area69.56ChemAxon
rotatable bond count36ChemAxon
refractivity193.38ChemAxon
polarizability86.09ChemAxon
Spectra
SpectraNot Available
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane
Biofluid Locations
  • Blood
Tissue Location
  • Brain
  • Fibroblasts
  • Intestine
  • Keratinocyte
  • Kidney
  • Liver
  • Muscle
  • Myelin
  • Nerve Cells
  • Neuron
  • Pancreas
  • Placenta
  • Platelet
  • Skeletal Muscle
  • Skin
  • Spleen
  • Stratum Corneum
  • Testes
  • Thyroid Gland
PathwaysNot Available
Normal Concentrations
BiofluidStatusValueAgeSexConditionReference
BloodDetected and Quantified0.022 +/- 0.001 uMAdult (>18 years old)BothNormal
BloodDetected and Quantified0.128 +/- 0.012 uMAdult (>18 years old)BothNormal
BloodDetected and Quantified0.040 +/- 0.002 uMAdult (>18 years old)BothNormal
BloodDetected and Quantified0.145 +/- 0.007 uMAdult (>18 years old)BothNormal
BloodDetected and Quantified1.22 +/- 0.046 uMAdult (>18 years old)BothNormal
BloodDetected and Quantified0.281 +/- 0.033 uMAdult (>18 years old)BothNormal
BloodDetected and Quantified1.00 +/- 0.029 uMAdult (>18 years old)BothNormal
BloodDetected and Quantified3.00 +/- 0.107 uMAdult (>18 years old)BothNormal
BloodDetected and Quantified0.271 +/- 0.013 uMAdult (>18 years old)BothNormal
BloodDetected and Quantified0.036 +/- 0.002 uMAdult (>18 years old)BothNormal
BloodDetected and Quantified0.061 +/- 0.0007 uMAdult (>18 years old)BothNormal
BloodDetected and Quantified0.012 +/- 0.001 uMAdult (>18 years old)BothNormal
BloodDetected and Quantified0.331 +/- 0.029 uMAdult (>18 years old)BothNormal
BloodDetected and Quantified0.008 +/- 0.001 uMAdult (>18 years old)BothNormal
Abnormal Concentrations
Not Available
Associated Disorders and Diseases
Disease ReferencesNone
Associated OMIM IDsNone
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB023539
KNApSAcK IDNot Available
Chemspider ID4446680
KEGG Compound IDC00195
BioCyc IDCERAMIDE
BiGG IDNot Available
Wikipedia LinkCer
NuGOwiki LinkHMDB04952
Metagene LinkHMDB04952
METLIN ID7205
PubChem Compound5283567
PDB IDNot Available
ChEBI IDNot Available
References
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Not Available
General References
  1. 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
  2. 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
  3. 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
  4. 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
  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
  6. 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
  7. 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
  8. 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
  9. 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
  10. 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
  11. 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
  12. 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
  13. 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
  14. 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
  15. 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
  16. 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
  17. 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
  18. 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
  19. 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
  20. 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
  21. 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
  22. 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
  23. 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
  24. 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
  25. 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
  26. 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
  27. 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
  28. 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
  29. Bowser PA, Gray GM: Sphingomyelinase in pig and human epidermis. J Invest Dermatol. 1978 Jun;70(6):331-5. Pubmed: 25935
  30. 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
  31. 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
  32. Humbert P: [Functional consequences of cutaneous lipid perturbation] Pathol Biol (Paris). 2003 Jul;51(5):271-4. Pubmed: 14567193
  33. 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
  34. 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
  35. 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
  36. 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
  37. 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
  38. 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
  39. 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
  40. 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
  41. 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
  42. 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
  43. Persaud-Sawin DA, Boustany RM: Cell death pathways in juvenile Batten disease. Apoptosis. 2005 Oct;10(5):973-85. Pubmed: 16151633
  44. 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
  45. 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
  46. 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
  47. 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
  48. 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
  49. 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
  50. 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
  51. 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
  52. 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
  53. Futerman AH: The roles of ceramide in the regulation of neuronal growth and development. Biochemistry (Mosc). 1998 Jan;63(1):74-83. Pubmed: 9526098
  54. 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
  55. Tserng KY, Griffin RL: Ceramide metabolite, not intact ceramide molecule, may be responsible for cellular toxicity. Biochem J. 2004 Jun 15;380(Pt 3):715-22. Pubmed: 14998372

Only showing the first 50 proteins. There are 70 proteins in total.

Enzymes

Gene Name:
PPAP2C
Uniprot ID:
O43688
Gene Name:
PPAP2A
Uniprot ID:
O14494
Gene Name:
PPAP2B
Uniprot ID:
O14495
Gene Name:
GALC
Uniprot ID:
P54803
Gene Name:
LCT
Uniprot ID:
P09848
Gene Name:
SMPD1
Uniprot ID:
P17405
Gene Name:
UGCG
Uniprot ID:
Q16739
Gene Name:
SGPL1
Uniprot ID:
O95470
Gene Name:
SPHK1
Uniprot ID:
Q9NYA1
Gene Name:
ASAH1
Uniprot ID:
Q13510
Gene Name:
SMPD2
Uniprot ID:
O60906
Gene Name:
COL4A3BP
Uniprot ID:
Q9Y5P4
Gene Name:
CERK
Uniprot ID:
Q8TCT0
Gene Name:
GM2A
Uniprot ID:
P17900
Gene Name:
GBA
Uniprot ID:
P04062
Gene Name:
NPC2
Uniprot ID:
P61916
Gene Name:
CERS1
Uniprot ID:
P27544
Gene Name:
CD1D
Uniprot ID:
P15813
Gene Name:
PIGM
Uniprot ID:
Q9H3S5
Gene Name:
PIGZ
Uniprot ID:
Q86VD9
Gene Name:
SMPD3
Uniprot ID:
Q9NY59
Gene Name:
B3GALT5
Uniprot ID:
Q9Y2C3
Gene Name:
PIGB
Uniprot ID:
Q92521
Gene Name:
PIGS
Uniprot ID:
Q96S52
Gene Name:
PIGT
Uniprot ID:
Q969N2
Gene Name:
PIGV
Uniprot ID:
Q9NUD9
Gene Name:
SMPD4
Uniprot ID:
Q9NXE4
Gene Name:
ASAH2C
Uniprot ID:
P0C7U2

Only showing the first 50 proteins. There are 70 proteins in total.