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Record Information
Version4.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2018-05-20 08:49:11 UTC
HMDB IDHMDB0001348
Secondary Accession Numbers
  • HMDB0012099
  • HMDB01348
  • HMDB12099
Metabolite Identification
Common NameSM(d18:1/18:0)
DescriptionSphingomyelin (d18:1/18:0) or SM(d18:1/18:0) is a type of sphingolipid found in animal cell membranes, especially in the membranous myelin sheath which surrounds some nerve cell axons. It usually consists of phosphorylcholine and ceramide. SM(18:1/18:0) consists of oleic acid attached to the C1 position and stearic acid attached to the C2 position. In humans, sphingomyelin is the only membrane phospholipid not derived from glycerol. Like all sphingolipids, SPH has a ceramide core (sphingosine bonded to a fatty acid via an amide linkage). In addition it contains one polar head group, which is either phosphocholine or phosphoethanolamine. The plasma membrane of cells is highly enriched in sphingomyelin and is considered largely to be found in the exoplasmic leaflet of the cell membrane. However, there is some evidence that there may also be a sphingomyelin pool in the inner leaflet of the membrane. Moreover, neutral sphingomyelinase-2 - an enzyme that breaks down sphingomyelin into ceramide has been found to localise exclusively to the inner leaflet further suggesting that there may be sphingomyelin present there. Sphingomyelin can accumulate in a rare hereditary disease called Niemann-Pick Disease, types A and B. Niemann-Pick disease is a genetically-inherited disease caused by a deficiency in the enzyme Sphingomyelinase, which causes the accumulation of Sphingomyelin in spleen, liver, lungs, bone marrow, and the brain, causing irreversible neurological damage. SMs play a role in signal transduction. Sphingomyelins are synthesized by the transfer of phosphorylcholine from phosphatidylcholine to a ceramide in a reaction catalyzed by sphingomyelin synthase.
Structure
Thumb
Synonyms
ValueSource
N-(Octadecanoyl)-sphing-4-enine-1-phosphocholineHMDB
N-Acyl-4-sphingenyl-1-O-phosphorylcholineHMDB
N-Acyl-D-sphingosine-1-phosphocholineHMDB
SphingomyelinHMDB
Sphingomyelin (D18:1/18:0)HMDB
Chemical FormulaC41H84N2O6P
Average Molecular Weight732.0892
Monoisotopic Molecular Weight731.606699942
IUPAC Name{[(2S,3R,4E)-3-hydroxy-2-octadecanamidooctadec-4-en-1-yl]oxy}[2-(trimethylazaniumyl)ethoxy]phosphinic acid
Traditional Name[(2S,3R,4E)-3-hydroxy-2-octadecanamidooctadec-4-en-1-yl]oxy(2-(trimethylammonio)ethoxy)phosphinic acid
CAS Registry Number85187-10-6
SMILES
CCCCCCCCCCCCCCCCCC(=O)N[C@@]([H])(COP(O)(=O)OCC[N+](C)(C)C)[C@]([H])(O)\C=C\CCCCCCCCCCCCC
InChI Identifier
InChI=1S/C41H83N2O6P/c1-6-8-10-12-14-16-18-20-21-23-25-27-29-31-33-35-41(45)42-39(38-49-50(46,47)48-37-36-43(3,4)5)40(44)34-32-30-28-26-24-22-19-17-15-13-11-9-7-2/h32,34,39-40,44H,6-31,33,35-38H2,1-5H3,(H-,42,45,46,47)/p+1/b34-32+/t39-,40+/m0/s1
InChI KeyLKQLRGMMMAHREN-YJFXYUILSA-O
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as phosphosphingolipids. These are sphingolipids with a structure based on a sphingoid base that is attached to a phosphate head group. They differ from phosphonospingolipids which have a phosphonate head group.
KingdomOrganic compounds
Super ClassLipids and lipid-like molecules
ClassSphingolipids
Sub ClassPhosphosphingolipids
Direct ParentPhosphosphingolipids
Alternative Parents
Substituents
  • Sphingoid-1-phosphate or derivatives
  • Phosphocholine
  • Phosphoethanolamine
  • Dialkyl phosphate
  • Fatty amide
  • N-acyl-amine
  • Organic phosphoric acid derivative
  • Phosphoric acid ester
  • Fatty acyl
  • Alkyl phosphate
  • Tetraalkylammonium salt
  • Quaternary ammonium salt
  • Secondary carboxylic acid amide
  • Secondary alcohol
  • Carboxamide group
  • Carboxylic acid derivative
  • Organic salt
  • Amine
  • Alcohol
  • Organic oxide
  • Organooxygen compound
  • Organonitrogen compound
  • Organopnictogen compound
  • Organic oxygen compound
  • Organic nitrogen compound
  • Hydrocarbon derivative
  • Carbonyl group
  • Organic cation
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External DescriptorsNot Available
Ontology
Physiological effect

Organoleptic effect:

Disposition

Route of exposure:

Source:

Biological location:

Process

Naturally occurring process:

Role

Industrial application:

Biological role:

Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility4.3e-05 g/LALOGPS
logP5.35ALOGPS
logP8.95ChemAxon
logS-7.2ALOGPS
pKa (Strongest Acidic)1.87ChemAxon
pKa (Strongest Basic)0.012ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count4ChemAxon
Hydrogen Donor Count3ChemAxon
Polar Surface Area105.09 ŲChemAxon
Rotatable Bond Count38ChemAxon
Refractivity224.27 m³·mol⁻¹ChemAxon
Polarizability93.09 ųChemAxon
Number of Rings0ChemAxon
Bioavailability0ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-001i-0200000900-e97fc8b60f9884266fe8View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-001i-0900000000-6e6f0a8e64b4641256a7View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-001i-0900000000-57c2d6df417e54414b35View in MoNA
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
2D NMR[1H,13C] 2D NMR SpectrumNot AvailableView in JSpectraViewer
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane
Biospecimen Locations
  • Blood
  • Breast Milk
  • Cerebrospinal Fluid (CSF)
  • Saliva
  • Urine
Tissue Location
  • Brain
  • Erythrocyte
  • Liver
  • Nervous Tissues
  • Spleen
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified6.90-38.0 uMAdult (>18 years old)Both
Normal
details
BloodDetected and Quantified1364 +/- 106 uMAdult (>18 years old)Female
Normal
details
BloodDetected and Quantified22.3 (18.6-25.8) uMNewborn (0-30 days old)Not Available
Normal
details
BloodDetected and Quantified298.0 +/- 24.0 uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified16.2 +/- 0.4 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified25.1 (21-30.9) uMInfant (0-1 year old)Not Available
Normal
details
BloodDetected and Quantified1145 +/- 67 uMAdult (>18 years old)Female
Normal
details
Breast MilkDetected and Quantified124 +/- 9 uMAdult (>18 years old)FemaleNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified0.340 +/- 0.154 uMAdult (>18 years old)Not SpecifiedNormal details
SalivaDetected and Quantified0.121 +/- 0.035 uMAdult (>18 years old)BothNormal
    • Zerihun T. Dame, ...
details
UrineDetected and Quantified0.0024 (0.0011-0.007) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified0.006 +/- 0.0144 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Mordechai, Hien, ...
details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified19.6 +/- 5.3 uMChildren (1-13 years old)Both
Obesity
    • Metabolomics reve...
details
BloodDetected and Quantified20.5 +/- 5.8 uMChildren (1-13 years old)Both
Obesity
    • Metabolomics reve...
details
UrineDetected and Quantified0.0028 +/- 0.0047 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Mordechai, Hien, ...
details
UrineDetected and Quantified0.0037 +/- 0.0062 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Gastroesophageal reflux disease
    • Mordechai, Hien, ...
details
Associated Disorders and Diseases
Disease References
Obesity
  1. Simone Wahl, Christina Holzapfel, Zhonghao Yu, Michaela Breier, Ivan Kondofersky, Christiane Fuchs, Paula Singmann, Cornelia Prehn, Jerzy Adamski, Harald Grallert, Thomas Illig, Rui Wang-Sattler, Thomas Reinehr (2013). Metabolomics reveals determinants of weight loss during lifestyle intervention in obese children. Metabolomics.
Eosinophilic esophagitis
  1. (). Mordechai, Hien, and David S. Wishart. .
Associated OMIM IDs
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FoodDB IDFDB022570
KNApSAcK IDNot Available
Chemspider ID4446701
KEGG Compound IDC00550
BioCyc IDSPHINGO-MYELIN
BiGG IDNot Available
Wikipedia LinkNot Available
METLIN ID176
PubChem Compound5283588
PDB IDNot Available
ChEBI ID17636
References
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. Haughey NJ, Cutler RG, Tamara A, McArthur JC, Vargas DL, Pardo CA, Turchan J, Nath A, Mattson MP: Perturbation of sphingolipid metabolism and ceramide production in HIV-dementia. Ann Neurol. 2004 Feb;55(2):257-67. [PubMed:14755730 ]
  2. Harzer K, Massenkeil G, Frohlich E: Concurrent increase of cholesterol, sphingomyelin and glucosylceramide in the spleen from non-neurologic Niemann-Pick type C patients but also patients possibly affected with other lipid trafficking disorders. FEBS Lett. 2003 Feb 27;537(1-3):177-81. [PubMed:12606053 ]
  3. He X, Chen F, McGovern MM, Schuchman EH: A fluorescence-based, high-throughput sphingomyelin assay for the analysis of Niemann-Pick disease and other disorders of sphingomyelin metabolism. Anal Biochem. 2002 Jul 1;306(1):115-23. [PubMed:12069422 ]
  4. Reichl D, Sterchi JM: Human peripheral lymph lipoproteins are enriched in sphingomyelin. Biochim Biophys Acta. 1992 Jul 9;1127(1):28-32. [PubMed:1627631 ]
  5. Nelson JC, Jiang XC, Tabas I, Tall A, Shea S: Plasma sphingomyelin and subclinical atherosclerosis: findings from the multi-ethnic study of atherosclerosis. Am J Epidemiol. 2006 May 15;163(10):903-12. Epub 2006 Apr 12. [PubMed:16611667 ]
  6. Chen H, Born E, Mathur SN, Johlin FC Jr, Field FJ: Sphingomyelin content of intestinal cell membranes regulates cholesterol absorption. Evidence for pancreatic and intestinal cell sphingomyelinase activity. Biochem J. 1992 Sep 15;286 ( Pt 3):771-7. [PubMed:1417735 ]
  7. Liu KZ, Mantsch HH: Simultaneous quantitation from infrared spectra of glucose concentrations, lactate concentrations, and lecithin/sphingomyelin ratios in amniotic fluid. Am J Obstet Gynecol. 1999 Mar;180(3 Pt 1):696-702. [PubMed:10076150 ]
  8. Horter MJ, Sondermann S, Reinecke H, Bogdanski J, Woltering A, Kerber S, Breithardt G, Assmann G, Von Eckardstein A: Associations of HDL phospholipids and paraoxonase activity with coronary heart disease in postmenopausal women. Acta Physiol Scand. 2002 Oct;176(2):123-30. [PubMed:12354172 ]
  9. Tanaka K, Nishizawa K, Yamamoto H, Naruto T, Izeki E, Taga T, Shimada M, Saeki Y: Analysis of very long-chain fatty acids and plasmalogen in the erythrocyte membrane: a simple method for the detection of peroxisomal disorders and discrimination between adrenoleukodystrophy and Zellweger syndrome. Neuropediatrics. 1990 Aug;21(3):119-23. [PubMed:2234315 ]
  10. Cribier S, Morrot G, Neumann JM, Devaux PF: Lateral diffusion of erythrocyte phospholipids in model membranes comparison between inner and outer leaflet components. Eur Biophys J. 1990;18(1):33-41. [PubMed:2155112 ]
  11. Nyberg L, Duan RD, Axelson J, Nilsson A: Identification of an alkaline sphingomyelinase activity in human bile. Biochim Biophys Acta. 1996 Mar 29;1300(1):42-8. [PubMed:8608160 ]
  12. Whitworth NS, Magann EF, Morrison JC: Evaluation of fetal lung maturity in diamniotic twins. Am J Obstet Gynecol. 1999 Jun;180(6 Pt 1):1438-41. [PubMed:10368484 ]
  13. de Oliveira JS, Zaharenko AJ, de Freitas JC, Konno K, de Andrade SA, Portaro FC, Richardson M, Sant'anna OA, Tambourgi DV: Caissarolysin I (Bcs I), a new hemolytic toxin from the Brazilian sea anemone Bunodosoma caissarum: purification and biological characterization. Biochim Biophys Acta. 2006 Mar;1760(3):453-61. Epub 2006 Jan 17. [PubMed:16458433 ]
  14. Omarini LP, Frank-Burkhardt SE, Seemayer TA, Mentha G, Terrier F: Niemann-Pick disease type C: nodular splenomegaly. Abdom Imaging. 1995 Mar-Apr;20(2):157-60. [PubMed:7787722 ]
  15. Berna L, Asfaw B, Conzelmann E, Cerny B, Ledvinova J: Determination of urinary sulfatides and other lipids by combination of reversed-phase and thin-layer chromatographies. Anal Biochem. 1999 May 1;269(2):304-11. [PubMed:10222002 ]
  16. He X, Chen F, Gatt S, Schuchman EH: An enzymatic assay for quantifying sphingomyelin in tissues and plasma from humans and mice with Niemann-Pick disease. Anal Biochem. 2001 Jun 15;293(2):204-11. [PubMed:11399033 ]
  17. Feki NC, Therond P, Couturier M, Limea G, Legrand A, Jouannet P, Auger J: Human sperm lipid content is modified after migration into human cervical mucus. Mol Hum Reprod. 2004 Feb;10(2):137-42. [PubMed:14742699 ]
  18. Wang C, Yang J, Gao P, Lu X, Xu G: Identification of phospholipid structures in human blood by direct-injection quadrupole-linear ion-trap mass spectrometry. Rapid Commun Mass Spectrom. 2005;19(17):2443-53. [PubMed:16059884 ]
  19. Otterbach B, Stoffel W: Acid sphingomyelinase-deficient mice mimic the neurovisceral form of human lysosomal storage disease (Niemann-Pick disease). Cell. 1995 Jun 30;81(7):1053-61. [PubMed:7600574 ]
  20. Fujiwaki T, Yamaguchi S, Tasaka M, Sakura N, Taketomi T: Application of delayed extraction-matrix-assisted laser desorption ionization time-of-flight mass spectrometry for analysis of sphingolipids in pericardial fluid, peritoneal fluid and serum from Gaucher disease patients. J Chromatogr B Analyt Technol Biomed Life Sci. 2002 Aug 25;776(1):115-23. [PubMed:12127332 ]

Only showing the first 10 proteins. There are 52 proteins in total.

Enzymes

General function:
Involved in transferase activity, transferring hexosyl groups
Specific function:
Catalyzes the formation of some glycolipid via the addition of N-acetylgalactosamine (GalNAc) in alpha-1,3-linkage to some substrate. Glycolipids probably serve for adherence of some pathogens
Gene Name:
GBGT1
Uniprot ID:
Q8N5D6
Molecular weight:
40126.9
General function:
Involved in N-acetylglucosaminylphosphatidylinositol de
Specific function:
Involved in the second step of GPI biosynthesis. De-N-acetylation of N-acetylglucosaminyl-phosphatidylinositol.
Gene Name:
PIGL
Uniprot ID:
Q9Y2B2
Molecular weight:
28530.965
General function:
Involved in catalytic activity
Specific function:
Bidirectional lipid cholinephosphotransferase capable of converting phosphatidylcholine (PC) and ceramide to sphingomyelin (SM) and diacylglycerol (DAG) and vice versa. Direction is dependent on the relative concentrations of DAG and ceramide as phosphocholine acceptors. Directly and specifically recognizes the choline head group on the substrate. Also requires two fatty chains on the choline-P donor molecule in order to be recognized efficiently as a substrate. Does not function strictly as a SM synthase. Required for cell growth
Gene Name:
SGMS2
Uniprot ID:
Q8NHU3
Molecular weight:
42279.8
General function:
Involved in catalytic activity
Specific function:
Bidirectional lipid cholinephosphotransferase capable of converting phosphatidylcholine (PC) and ceramide to sphingomyelin (SM) and diacylglycerol (DAG) and vice versa. Direction is dependent on the relative concentrations of DAG and ceramide as phosphocholine acceptors. Directly and specifically recognizes the choline head group on the substrate. Also requires two fatty chains on the choline-P donor molecule in order to be recognized efficiently as a substrate. Does not function strictly as a SM synthase. Suppresses BAX-mediated apoptosis and also prevents cell death in response to stimuli such as hydrogen peroxide, osmotic stress, elevated temperature and exogenously supplied sphingolipids. May protect against cell death by reversing the stress-inducible increase in levels of proapoptotic ceramide. Required for cell growth
Gene Name:
SGMS1
Uniprot ID:
Q86VZ5
Molecular weight:
49207.3
General function:
Involved in hydrolase activity
Specific function:
Converts sphingomyelin to ceramide. Also has phospholipase C activities toward 1,2-diacylglycerolphosphocholine and 1,2-diacylglycerolphosphoglycerol. Isoform 2 and isoform 3 have lost catalytic activity.
Gene Name:
SMPD1
Uniprot ID:
P17405
Molecular weight:
69935.53
General function:
Involved in galactosylgalactosylxylosylprotein 3-beta-glucuronosyltransferase activity
Specific function:
Involved in the biosynthesis of L2/HNK-1 carbohydrate epitope on glycoproteins. Can also play a role in glycosaminoglycan biosynthesis. Substrates include asialo-orosomucoid (ASOR), asialo-fetuin, and asialo-neural cell adhesion molecule. Requires sphingomyelin for activity: stearoyl-sphingomyelin was the most effective, followed by palmitoyl-sphingomyelin and lignoceroyl-sphingomyelin. Activity was demonstrated only for sphingomyelin with a saturated fatty acid and not for that with an unsaturated fatty acid, regardless of the length of the acyl group (By similarity).
Gene Name:
B3GAT1
Uniprot ID:
Q9P2W7
Molecular weight:
38255.675
General function:
Involved in phosphatidylinositol N-acetylglucosaminyltransferase activity
Specific function:
Part of the complex catalyzing the transfer of N-acetylglucosamine from UDP-N-acetylglucosamine to phosphatidylinositol, the first step of GPI biosynthesis.
Gene Name:
PIGQ
Uniprot ID:
Q9BRB3
Molecular weight:
65343.25
General function:
Involved in biosynthetic process
Specific function:
Necessary for the synthesis of N-acetylglucosaminyl-phosphatidylinositol, the very early intermediate in GPI-anchor biosynthesis.
Gene Name:
PIGA
Uniprot ID:
P37287
Molecular weight:
54126.065
General function:
Involved in phosphatidylinositol N-acetylglucosaminyltr
Specific function:
Part of the complex catalyzing the transfer of N-acetylglucosamine from UDP-N-acetylglucosamine to phosphatidylinositol, the first step of GPI biosynthesis.
Gene Name:
PIGH
Uniprot ID:
Q14442
Molecular weight:
21080.415
General function:
Involved in phosphatidylinositol N-acetylglucosaminyltr
Specific function:
Part of the complex catalyzing the transfer of N-acetylglucosamine from UDP-N-acetylglucosamine to phosphatidylinositol, the first step of GPI biosynthesis.
Gene Name:
PIGP
Uniprot ID:
P57054
Molecular weight:
18089.055

Only showing the first 10 proteins. There are 52 proteins in total.