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Record Information
Version4.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2018-04-16 16:59:18 UTC
HMDB IDHMDB0000054
Secondary Accession Numbers
  • HMDB00054
Metabolite Identification
Common NameBilirubin
DescriptionBilirubin is a bile pigment that is a degradation product of heme. In particular, bilirubin is a yellow breakdown product of normal heme catabolism. Its levels are elevated in certain diseases and it is responsible for the yellow colour of bruises. Bilirubin is an excretion product and the body does not control its levels. Bilirubin levels reflect the balance between production and excretion. Thus, there is no "normal" level of bilirubin. Bilirubin consists of an open chain of four pyrroles (tetrapyrrole). In contrast, the heme molecule is a closed ring of four pyrroles, called porphyrin (Wikipedia). Bilirubin is found to be associated with Crigler-Najjar syndrome type I, which is an inborn error of metabolism.
Structure
Thumb
Synonyms
ValueSource
1,10,19,22,23,24-hexahydro-2,7,13,17-Tetramethyl-1,19-dioxo-3,18-divinylbiline-8,12-dipropionic acidChEBI
2,17-Diethenyl-1,10,19,22,23,24-hexahydro-3,7,13,18-tetramethyl-1,19-dioxo-21H-biline-8,12-dipropanoic acidChEBI
2,7,13,17-Tetramethyl-1,19-dioxo-3,18-divinyl-1,10,19,22,23,24-hexahydro-21H-biline-8,12-dipropanoic acidChEBI
8,12-Bis(2-carboxyethyl)-2,7,13,17-tetramethyl-3,18-divinylbiladiene-ac-1,19(21H,24H)-dioneChEBI
Bilirubin ixalphaChEBI
1,10,19,22,23,24-hexahydro-2,7,13,17-Tetramethyl-1,19-dioxo-3,18-divinylbiline-8,12-dipropionateGenerator
2,17-Diethenyl-1,10,19,22,23,24-hexahydro-3,7,13,18-tetramethyl-1,19-dioxo-21H-biline-8,12-dipropanoateGenerator
2,7,13,17-Tetramethyl-1,19-dioxo-3,18-divinyl-1,10,19,22,23,24-hexahydro-21H-biline-8,12-dipropanoateGenerator
(4Z,15Z)-Bilirubin ixaHMDB
(Z,Z)-Bilirubin ixaHMDB
1,10,19,22,23,24-hexahydro-2,7,13,17-Tetramethyl-1,19-dioxo-3,18-divinyl-biline-8,12-dipropionateHMDB
1,10,19,22,23,24-hexahydro-2,7,13,17-Tetramethyl-1,19-dioxo-3,18-divinyl-biline-8,12-dipropionic acidHMDB
3-(2-((3-(2-Carboxyethyl)-4-methyl-5-((3-methyl-5-oxo-4-vinyl-1,5-dihydro-2H-pyrrol-2-ylidene)methyl)-1H-pyrrol-2-yl)methyl)-4-methyl-5-((4-methyl-5-oxo-3-vinyl-1,5-dihydro-2H-pyrrol-2-ylidene)methyl)-1H-pyrrol-3-yl)propanoateHMDB
3-(2-((3-(2-Carboxyethyl)-4-methyl-5-((3-methyl-5-oxo-4-vinyl-1,5-dihydro-2H-pyrrol-2-ylidene)methyl)-1H-pyrrol-2-yl)methyl)-4-methyl-5-((4-methyl-5-oxo-3-vinyl-1,5-dihydro-2H-pyrrol-2-ylidene)methyl)-1H-pyrrol-3-yl)propanoic acidHMDB
3-(2-((3-(2-Carboxyethyl)-4-methyl-5-[(Z)-(3-methyl-5-oxo-4-vinyl-1,5-dihydro-2H-pyrrol-2-ylidene)methyl]-1H-pyrrol-2-yl)methyl)-4-methyl-5-[(Z)-(4-methyl-5-oxo-3-vinyl-1,5-dihydro-2H-pyrrol-2-ylideneHMDB
3-[2-[[3-(2-Carboxyethyl)-5-[(3-ethenyl-4-methyl-5-oxo-pyrrol-2-ylidene)methyl]-4-methyl-1H-pyrrol-2-yl]methyl]-5-[(4-ethenyl-3-methyl-5-oxo-pyrrol-2-ylidene)methyl]-4-methyl-1H-pyrrol-3-yl]propanoateHMDB
3-[2-[[3-(2-Carboxyethyl)-5-[(3-ethenyl-4-methyl-5-oxo-pyrrol-2-ylidene)methyl]-4-methyl-1H-pyrrol-2-yl]methyl]-5-[(4-ethenyl-3-methyl-5-oxo-pyrrol-2-ylidene)methyl]-4-methyl-1H-pyrrol-3-yl]propanoic acidHMDB
3-[2-[[3-(2-Carboxyethyl)-5-[(Z)-(3-ethenyl-4-methyl-5-oxo-pyrrol-2-ylidene)methyl]-4-methyl-1H-pyrrol-2-yl]methyl]-5-[(Z)-(4-ethenyl-3-methyl-5-oxo-pyrrol-2-ylidene)methyl]-4-methyl-1H-pyrrol-3-yl]propanoateHMDB
3-[2-[[3-(2-Carboxyethyl)-5-[(Z)-(3-ethenyl-4-methyl-5-oxo-pyrrol-2-ylidene)methyl]-4-methyl-1H-pyrrol-2-yl]methyl]-5-[(Z)-(4-ethenyl-3-methyl-5-oxo-pyrrol-2-ylidene)methyl]-4-methyl-1H-pyrrol-3-yl]propanoic acidHMDB
Bilirubin IX-alphaHMDB
CholerythrinHMDB
HematoidinHMDB
Bilirubin IX alphaMeSH
Disodium salt bilirubinMeSH
Bilirubin, calcium saltMeSH
Calcium salt bilirubinMeSH
delta-BilirubinMeSH
Bilirubin, (4E)-isomerMeSH
Bilirubin, (4E,15E)-isomerMeSH
Bilirubin, disodium saltMeSH
Bilirubin, monosodium saltMeSH
Calcium bilirubinateMeSH
Salt bilirubin, calciumMeSH
Bilirubin, (15E)-isomerMeSH
Bilirubinate, calciumMeSH
Monosodium salt bilirubinMeSH
delta BilirubinMeSH
Chemical FormulaC33H36N4O6
Average Molecular Weight584.6621
Monoisotopic Molecular Weight584.263484904
IUPAC Name3-(2-{[3-(2-carboxyethyl)-5-{[(2Z)-4-ethenyl-3-methyl-5-oxo-2,5-dihydro-1H-pyrrol-2-ylidene]methyl}-4-methyl-1H-pyrrol-2-yl]methyl}-5-{[(2Z)-3-ethenyl-4-methyl-5-oxo-2,5-dihydro-1H-pyrrol-2-ylidene]methyl}-4-methyl-1H-pyrrol-3-yl)propanoic acid
Traditional Namebilirubin
CAS Registry Number635-65-4
SMILES
CC1=C(C=C)\C(NC1=O)=C\C1=C(C)C(CCC(O)=O)=C(CC2=C(CCC(O)=O)C(C)=C(N2)\C=C2/NC(=O)C(C=C)=C2C)N1
InChI Identifier
InChI=1S/C33H36N4O6/c1-7-20-19(6)32(42)37-27(20)14-25-18(5)23(10-12-31(40)41)29(35-25)15-28-22(9-11-30(38)39)17(4)24(34-28)13-26-16(3)21(8-2)33(43)36-26/h7-8,13-14,34-35H,1-2,9-12,15H2,3-6H3,(H,36,43)(H,37,42)(H,38,39)(H,40,41)/b26-13-,27-14-
InChI KeyBPYKTIZUTYGOLE-IFADSCNNSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as bilirubins. These are organic compounds containing a dicarboxylic acyclic tetrapyrrole derivative.
KingdomOrganic compounds
Super ClassOrganoheterocyclic compounds
ClassTetrapyrroles and derivatives
Sub ClassBilirubins
Direct ParentBilirubins
Alternative Parents
Substituents
  • Bilirubin skeleton
  • Dicarboxylic acid or derivatives
  • Substituted pyrrole
  • Pyrrole
  • Pyrroline
  • Heteroaromatic compound
  • Secondary carboxylic acid amide
  • Lactam
  • Carboxamide group
  • Azacycle
  • Carboxylic acid
  • Carboxylic acid derivative
  • Organopnictogen compound
  • Organic nitrogen compound
  • Organooxygen compound
  • Organonitrogen compound
  • Organic oxygen compound
  • Carbonyl group
  • Hydrocarbon derivative
  • Organic oxide
  • Aromatic heteromonocyclic compound
Molecular FrameworkAromatic heteromonocyclic compounds
External Descriptors
Ontology
Physiological effect

Health effect:

Disposition

Route of exposure:

Source:

Biological location:

Process

Naturally occurring process:

Role

Biological role:

Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water Solubility0.009 mg/mL at 25 °CNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility0.0096 g/LALOGPS
logP3.22ALOGPS
logP3.12ChemAxon
logS-4.8ALOGPS
pKa (Strongest Acidic)4.03ChemAxon
pKa (Strongest Basic)-2.8ChemAxon
Physiological Charge-2ChemAxon
Hydrogen Acceptor Count6ChemAxon
Hydrogen Donor Count6ChemAxon
Polar Surface Area164.38 ŲChemAxon
Rotatable Bond Count12ChemAxon
Refractivity168.9 m³·mol⁻¹ChemAxon
Polarizability66.16 ųChemAxon
Number of Rings4ChemAxon
Bioavailability0ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-00vr-0100090000-d19585e898437e8be1d5View in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (1 TMS) - 70eV, Positivesplash10-01vn-3100069000-c39a7113e7f8eb3fa22aView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-IT , negativesplash10-000i-0090000000-50ff729317f07f6120b5View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-00ks-0000090000-ddf9acb7f968b9079764View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-00g0-0110290000-ae6f323e9f5e9e26199cView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-0uml-1102910000-df5146b3612e4fb4ce5eView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-001i-0000090000-6deb1aed1a9c3839e2a9View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-053i-1010190000-7435a4388bade8e83d10View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-052f-9010240000-cc90fff0581c6bcefd0dView in MoNA
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR13C NMR SpectrumNot AvailableView in JSpectraViewer
2D NMR[1H,13C] 2D NMR SpectrumNot AvailableView in JSpectraViewer
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Membrane (predicted from logP)
  • Endoplasmic reticulum
Biospecimen Locations
  • Bile
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Urine
Tissue Location
  • Bile Duct
  • Bone Marrow
  • Brain
  • Erythrocyte
  • Fibroblasts
  • Intestine
  • Kidney
  • Liver
  • Lymph Node
  • Muscle
  • Myelin
  • Nerve Cells
  • Neuron
  • Pancreas
  • Placenta
  • Platelet
  • Reticulocyte
  • Skin
  • Spleen
  • Thyroid Gland
Pathways
NameSMPDB/PathwhizKEGG
Acute Intermittent PorphyriaThumbThumb?image type=greyscaleThumb?image type=simpleNot Available
Congenital Erythropoietic Porphyria (CEP) or Gunther DiseaseThumbThumb?image type=greyscaleThumb?image type=simpleNot Available
Hereditary Coproporphyria (HCP)ThumbThumb?image type=greyscaleThumb?image type=simpleNot Available
Porphyria Variegata (PV)ThumbThumb?image type=greyscaleThumb?image type=simpleNot Available
Porphyrin MetabolismThumbThumb?image type=greyscaleThumb?image type=simpleMap00860
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified13.0 (5.0-21.0) uMAdult (>18 years old)Both
Normal
    • McPherson, Richar...
    • Louise M. Malark...
details
BloodDetected and Quantified15.2 +/- 2.0 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified<12.828 uMAdult (>18 years old)Both
Normal
details
BloodDetected and Quantified<12 uMNewborn (0-30 days old)Not Specified
Normal
details
BloodDetected and Quantified<3.4 uMChildren (1-13 years old)Not Specified
Normal
details
BloodDetected and Quantified3-21 uMNewborn (0-30 days old)Not Specified
Normal
details
BloodDetected and Quantified<20 uMChildren (1-13 years old)Not Specified
Normal
details
BloodDetected and Quantified8.0 +/- 0.9 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified310.265 +/- 65.850 uMNewborn (0-30 days old)Not SpecifiedNormal details
BloodDetected and Quantified6.842-20.525 uMChildren (1-13 years old)Not Specified
Normal
details
BloodDetected and Quantified5.131-17.104 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified5.10-18.0 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified<17 uMChildren (1 - 13 years old)Not AvailableNormal
    • Hereditary Jaundi...
details
BloodDetected and Quantified8.500 +/- 4 uMAdult (>18 years old)Not SpecifiedNormal
    • Geigy Scientific ...
details
BloodDetected but not Quantified Adult (>18 years old)Both
Normal
details
Cerebrospinal Fluid (CSF)Detected and Quantified0 - 0.2 uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
UrineDetected and Quantified< 0.1 umol/mmol creatinineAdult (>18 years old)BothNormal
    • Tietz Clinical Gu...
details
UrineDetected and Quantified0.032 (0.0019-0.21) umol/mmol creatinineAdult (>18 years old)BothNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
details
UrineDetected and Quantified0.51-5.13 umol/mmol creatinineAdult (>18 years old)BothNormal
    • David F. Putnam C...
details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BileDetected and Quantified5000 (4300-5700) uMAdult (>18 years old)BothCrohn's disease details
BileDetected and Quantified2600 (2400-2800) uMAdult (>18 years old)BothGallstone disease details
BileDetected and Quantified4600 (3900-5300) uMAdult (>18 years old)Both
Crohn's disease
details
BileDetected and Quantified5900 (4300-7500) uMAdult (>18 years old)Both
Crohn's disease
details
BloodDetected and Quantified14.6 +/- 2.5 uMAdult (>18 years old)Both
Growth hormone deficiency
details
BloodDetected and Quantified650 uMAdolescent (13-18 years old)MaleCrigler-Najjar syndrome Type I details
BloodDetected and Quantified25.656-78.678 uMAdult (>18 years old)Both
Anemia, congenital dyserythropoietic, type II
details
BloodDetected and Quantified28.7-170 uMChildren (1-13 years old)Both
Infantile Liver Failure Syndrome 2
details
BloodDetected and Quantified22-41 uMNewborn (0-30 days old)Both
Citrullinemia type II, neonatal-onset
details
BloodDetected and Quantified46.9-233 uMChildren (1-13 years old)Both
Infantile Liver Failure Syndrome 2
details
BloodDetected and Quantified41-101 uMNewborn (0-30 days old)Both
Citrullinemia type II, neonatal-onset
details
BloodDetected and Quantified73.1-85.0 uMInfant (0-1 year old)Female
Infantile Liver Failure Syndrome 2
details
BloodDetected and Quantified41(6-381) uMChildren (1-13 years old)BothCholestasis, progressive familial intrahepatic, 1 details
BloodDetected and Quantified59.2 uMInfant (0-1 year old)Female
Wolcott-Rallison syndrome
    • Neonatal diabetes...
details
BloodDetected and Quantified106 uMInfant (0-1 year old)FemaleBile Acid Synthesis Defect, Congenital, 1 details
BloodDetected and Quantified24.5 uMAdult (>18 years old)FemaleLecithin:cholesterol Acyltransferase Deficiency details
BloodDetected and Quantified8.80 +/- 1.22 uMAdult (>18 years old)Both
Growth hormone deficiency
details
BloodDetected and Quantified573.0 (291.0-855.0) uMChildren (1-13 years old)BothCrigler-Najjar syndrome Type I
    • MetaGene: Metabol...
details
BloodDetected and Quantified29.59 uMNewborn (0-30 days old)Male
Lathosterolosis
details
BloodDetected and Quantified380.733 +/- 142.989 uMNewborn (0-30 days old)Both
Glucose-6-phosphate dehydrogenase deficiency
details
BloodDetected and Quantified6.842-20.525 uMAdult (>18 years old)Male
Primary Hypomagnesemia
details
BloodDetected and Quantified18.814-518.248 uMAdult (>18 years old)Male
Cholestasis, progressive familial intrahepatic, 1
details
BloodDetected and Quantified42.247 uMNewborn (0-30 days old)Male
Lathosterolosis
details
BloodDetected and Quantified42.760-141.962 uMChildren (1-13 years old)Female
Cholestasis, progressive familial intrahepatic, 1
details
BloodDetected and Quantified78.678 uMNewborn (0-30 days old)MaleDonohue Syndrome details
BloodDetected and Quantified111.175 uMNewborn (0-30 days old)MaleDonohue Syndrome details
Cerebrospinal Fluid (CSF)Detected and Quantified17.3 +/- 12.4 uMAdult (>18 years old)Both
Cerebral Vasospasm
details
FecesDetected but not Quantified Adult (>18 years old)Both
Colorectal cancer
details
FecesDetected but not Quantified Newborn (0-30 days old)Not Specified
Premature neonates
details
FecesDetected but not Quantified Adult (>18 years old)BothColorectal Cancer details
Associated Disorders and Diseases
Disease References
Cerebral vasospasm
  1. Pyne-Geithman GJ, Morgan CJ, Wagner K, Dulaney EM, Carrozzella J, Kanter DS, Zuccarello M, Clark JF: Bilirubin production and oxidation in CSF of patients with cerebral vasospasm after subarachnoid hemorrhage. J Cereb Blood Flow Metab. 2005 Aug;25(8):1070-7. [PubMed:15789034 ]
Crohn's disease
  1. Lapidus A, Akerlund JE, Einarsson C: Gallbladder bile composition in patients with Crohn 's disease. World J Gastroenterol. 2006 Jan 7;12(1):70-4. [PubMed:16440420 ]
Gallbladder disease
  1. Lapidus A, Akerlund JE, Einarsson C: Gallbladder bile composition in patients with Crohn 's disease. World J Gastroenterol. 2006 Jan 7;12(1):70-4. [PubMed:16440420 ]
Crigler-Najjar syndrome type I
  1. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Anemia, congenital dyserythropoietic, type II
  1. Bianchi P, Fermo E, Vercellati C, Boschetti C, Barcellini W, Iurlo A, Marcello AP, Righetti PG, Zanella A: Congenital dyserythropoietic anemia type II (CDAII) is caused by mutations in the SEC23B gene. Hum Mutat. 2009 Sep;30(9):1292-8. doi: 10.1002/humu.21077. [PubMed:19621418 ]
Bile Acid Synthesis Defect, Congenital, 1
  1. Huang HY, Zhou H, Wang H, Chen YX, Fang F: Novel Mutations in the 3beta-hydroxy-5-C27-steroid Dehydrogenase Gene (HSD3B7) in a Patient with Neonatal Cholestasis. Chin Med J (Engl). 2016 Jan 5;129(1):98-100. doi: 10.4103/0366-6999.172603. [PubMed:26712441 ]
Cholestasis, progressive familial intrahepatic, 1
  1. Nagasaka H, Yorifuji T, Kosugiyama K, Egawa H, Kawai M, Murayama K, Hasegawa M, Sumazaki R, Tsubaki J, Kikuta H, Matsui A, Tanaka K, Matsuura N, Kobayashi K: Resistance to parathyroid hormone in two patients with familial intrahepatic cholestasis: possible involvement of the ATP8B1 gene in calcium regulation via parathyroid hormone. J Pediatr Gastroenterol Nutr. 2004 Oct;39(4):404-9. [PubMed:15448432 ]
  2. Schukfeh N, Metzelder ML, Petersen C, Reismann M, Pfister ED, Ure BM, Kuebler JF: Normalization of serum bile acids after partial external biliary diversion indicates an excellent long-term outcome in children with progressive familial intrahepatic cholestasis. J Pediatr Surg. 2012 Mar;47(3):501-5. doi: 10.1016/j.jpedsurg.2011.08.010. [PubMed:22424345 ]
Citrullinemia type II, neonatal-onset
  1. Ohura T, Kobayashi K, Tazawa Y, Nishi I, Abukawa D, Sakamoto O, Iinuma K, Saheki T: Neonatal presentation of adult-onset type II citrullinemia. Hum Genet. 2001 Feb;108(2):87-90. [PubMed:11281457 ]
Donohue Syndrome
  1. Nijim Y, Awni Y, Adawi A, Bowirrat A: Classic Case Report of Donohue Syndrome (Leprechaunism; OMIM *246200): The Impact of Consanguineous Mating. Medicine (Baltimore). 2016 Feb;95(6):e2710. doi: 10.1097/MD.0000000000002710. [PubMed:26871809 ]
Glucose-6-phosphate dehydrogenase deficiency
  1. Iranpour R, Akbar MR, Haghshenas I: Glucose-6-phosphate dehydrogenase deficiency in neonates. Indian J Pediatr. 2003 Nov;70(11):855-7. [PubMed:14703221 ]
Infantile Liver Failure Syndrome 2
  1. Staufner C, Haack TB, Kopke MG, Straub BK, Kolker S, Thiel C, Freisinger P, Baric I, McKiernan PJ, Dikow N, Harting I, Beisse F, Burgard P, Kotzaeridou U, Lenz D, Kuhr J, Himbert U, Taylor RW, Distelmaier F, Vockley J, Ghaloul-Gonzalez L, Ozolek JA, Zschocke J, Kuster A, Dick A, Das AM, Wieland T, Terrile C, Strom TM, Meitinger T, Prokisch H, Hoffmann GF: Recurrent acute liver failure due to NBAS deficiency: phenotypic spectrum, disease mechanisms, and therapeutic concepts. J Inherit Metab Dis. 2016 Jan;39(1):3-16. doi: 10.1007/s10545-015-9896-7. Epub 2015 Nov 5. [PubMed:26541327 ]
Lathosterolosis
  1. Brunetti-Pierri N, Corso G, Rossi M, Ferrari P, Balli F, Rivasi F, Annunziata I, Ballabio A, Russo AD, Andria G, Parenti G: Lathosterolosis, a novel multiple-malformation/mental retardation syndrome due to deficiency of 3beta-hydroxysteroid-delta5-desaturase. Am J Hum Genet. 2002 Oct;71(4):952-8. Epub 2002 Aug 20. [PubMed:12189593 ]
Lecithin:cholesterol Acyltransferase Deficiency
  1. Idzior-Walus B, Sieradzki J, Kostner G, Malecki MT, Klupa T, Wesolowska T, Rostworowski W, Hartwich J, Walus M, Kiec AD, Naruszewicz M: Familial lecithin-cholesterol acyltransferase deficiency: biochemical characteristics and molecular analysis of a new LCAT mutation in a Polish family. Atherosclerosis. 2006 Apr;185(2):413-20. Epub 2005 Jul 26. [PubMed:16051254 ]
Wolcott-Rallison syndrome
  1. Can Thi Bich Ngoc, Vu Chi Dung, Sarah Flanagan and Sian Ellard (2013). Neonatal diabetes in Wolcott-Rallison syndrome: a case report. International Journal of Pediatric Endocrinology.
Associated OMIM IDs
  • 266600 (Crohn's disease)
  • 218800 (Crigler-Najjar syndrome type I)
  • 224100 (Anemia, congenital dyserythropoietic, type II)
  • 607330 (Lathosterolosis)
  • 300908 (Glucose-6-phosphate dehydrogenase deficiency)
  • 211600 (Cholestasis, progressive familial intrahepatic, 1)
  • 246200 (Donohue Syndrome)
  • 605814 (Citrullinemia type II, neonatal-onset)
  • 616483 (Infantile Liver Failure Syndrome 2)
  • 607765 (Bile Acid Synthesis Defect, Congenital, 1)
  • 245900 (Lecithin:cholesterol Acyltransferase Deficiency)
  • 226980 (Wolcott-Rallison syndrome)
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FoodDB IDFDB021886
KNApSAcK IDC00029828
Chemspider ID4444055
KEGG Compound IDC00486
BioCyc IDBILIRUBIN
BiGG ID35117
Wikipedia LinkBilirubin
METLIN ID81
PubChem Compound5280352
PDB IDNot Available
ChEBI ID16990
References
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. Randeberg LL, Roll EB, Nilsen LT, Christensen T, Svaasand LO: In vivo spectroscopy of jaundiced newborn skin reveals more than a bilirubin index. Acta Paediatr. 2005 Jan;94(1):65-71. [PubMed:15858963 ]
  2. Bayes Garcia R, Maldonado Lozano J, Molina Font JA: [Interrelation of bilirubin and free fatty acids in newborn infants with pathologic conditions]. An Esp Pediatr. 1989 Jan;30(1):27-31. [PubMed:2648917 ]
  3. Yamamoto S, Kubo S, Hai S, Uenishi T, Yamamoto T, Shuto T, Takemura S, Tanaka H, Yamazaki O, Hirohashi K, Tanaka T: Hepatitis C virus infection as a likely etiology of intrahepatic cholangiocarcinoma. Cancer Sci. 2004 Jul;95(7):592-5. [PubMed:15245596 ]
  4. Kabicek P: Importance of serum bile acids determination in adolescents with juvenile hyperbilirubinaemia. Cent Eur J Public Health. 2004 Jun;12(2):102-9. [PubMed:15242029 ]
  5. Tiribelli C, Ostrow JD: New concepts in bilirubin and jaundice: report of the Third International Bilirubin Workshop, April 6-8, 1995, Trieste, Italy. Hepatology. 1996 Nov;24(5):1296-311. [PubMed:8903413 ]
  6. Zhan X, Wang SY, Wang L, Qu P: [Decreased peripheral nerve conduction velocity may be associated with lower-serum level of vitamin E in patients with infantile hepatitis syndrome]. Zhonghua Er Ke Za Zhi. 2004 May;42(5):362-6. [PubMed:15189696 ]
  7. Deja M, Hildebrandt B, Ahlers O, Riess H, Wust P, Gerlach H, Kerner T: Goal-directed therapy of cardiac preload in induced whole-body hyperthermia. Chest. 2005 Aug;128(2):580-6. [PubMed:16100141 ]
  8. Kikuchi S, Hata M, Fukumoto K, Yamane Y, Matsui T, Tamura A, Yonemura S, Yamagishi H, Keppler D, Tsukita S, Tsukita S: Radixin deficiency causes conjugated hyperbilirubinemia with loss of Mrp2 from bile canalicular membranes. Nat Genet. 2002 Jul;31(3):320-5. Epub 2002 Jun 17. [PubMed:12068294 ]
  9. Lin JM, Jiang CQ: [Clinical manifestation and ultrasonic characteristics of five patients with acute arsenic poisoning]. Zhonghua Lao Dong Wei Sheng Zhi Ye Bing Za Zhi. 2003 Dec;21(6):420-2. [PubMed:14761351 ]
  10. Azer SA: A multimedia CD-ROM tool to improve student understanding of bile salts and bilirubin metabolism: evaluation of its use in a medical hybrid PBL course. Adv Physiol Educ. 2005 Mar;29(1):40-50. [PubMed:15718382 ]
  11. Slusher TM, Angyo IA, Bode-Thomas F, Akor F, Pam SD, Adetunji AA, McLaren DW, Wong RJ, Vreman HJ, Stevenson DK: Transcutaneous bilirubin measurements and serum total bilirubin levels in indigenous African infants. Pediatrics. 2004 Jun;113(6):1636-41. [PubMed:15173484 ]
  12. Ciszowski K, Gomolka E, Jenner B: [The influence of the dose, time since ingestion and concentration of the xenobiotic on the clinical state and severity of liver damage with patients intoxicated with paracetamol]. Przegl Lek. 2005;62(6):456-61. [PubMed:16225094 ]
  13. Sando M, Sato Y, Iwata S, Akita H, Sunakawa K: In vitro protein binding of teicoplanin to neonatal serum. J Infect Chemother. 2004 Oct;10(5):280-3. [PubMed:16163462 ]
  14. Danko I, Jia Z, Zhang G: Nonviral gene transfer into liver and muscle for treatment of hyperbilirubinemia in the gunn rat. Hum Gene Ther. 2004 Dec;15(12):1279-86. [PubMed:15684703 ]
  15. Kotal P, Van der Veere CN, Sinaasappel M, Elferink RO, Vitek L, Brodanova M, Jansen PL, Fevery J: Intestinal excretion of unconjugated bilirubin in man and rats with inherited unconjugated hyperbilirubinemia. Pediatr Res. 1997 Aug;42(2):195-200. [PubMed:9262222 ]
  16. Ochenashko OV, Volkova NA, Mazur SP, Somov AY, Fuller BJ, Petrenko AY: Cryopreserved fetal liver cell transplants support the chronic failing liver in rats with CCl4-induced cirrhosis. Cell Transplant. 2006;15(1):23-33. [PubMed:16700327 ]
  17. Lapidus A, Akerlund JE, Einarsson C: Gallbladder bile composition in patients with Crohn 's disease. World J Gastroenterol. 2006 Jan 7;12(1):70-4. [PubMed:16440420 ]
  18. Sikkel E, Pasman SA, Oepkes D, Kanhai HH, Vandenbussche FP: On the origin of amniotic fluid bilirubin. Placenta. 2004 May;25(5):463-8. [PubMed:15081641 ]
  19. Schmidt CM, Powell ES, Yiannoutsos CT, Howard TJ, Wiebke EA, Wiesenauer CA, Baumgardner JA, Cummings OW, Jacobson LE, Broadie TA, Canal DF, Goulet RJ Jr, Curie EA, Cardenes H, Watkins JM, Loehrer PJ, Lillemoe KD, Madura JA: Pancreaticoduodenectomy: a 20-year experience in 516 patients. Arch Surg. 2004 Jul;139(7):718-25; discussion 725-7. [PubMed:15249403 ]
  20. Nanjundaswamy S, Petrova A, Mehta R, Hegyi T: Transcutaneous bilirubinometry in preterm infants receiving phototherapy. Am J Perinatol. 2005 Apr;22(3):127-31. [PubMed:15838745 ]

Only showing the first 10 proteins. There are 28 proteins in total.

Enzymes

General function:
Involved in heme oxygenase (decyclizing) activity
Specific function:
Heme oxygenase cleaves the heme ring at the alpha methene bridge to form biliverdin. Biliverdin is subsequently converted to bilirubin by biliverdin reductase. Under physiological conditions, the activity of heme oxygenase is highest in the spleen, where senescent erythrocytes are sequestrated and destroyed.
Gene Name:
HMOX1
Uniprot ID:
P09601
Molecular weight:
32818.345
References
  1. Matsuoka Y, Masuda H, Yokoyama M, Kihara K: Protective effects of bilirubin against cyclophosphamide induced hemorrhagic cystitis in rats. J Urol. 2008 Mar;179(3):1160-6. doi: 10.1016/j.juro.2007.10.031. [PubMed:18206168 ]
General function:
Involved in transferase activity, transferring hexosyl groups
Specific function:
UDPGTs are of major importance in the conjugation and subsequent elimination of potentially toxic xenobiotics and endogenous compounds. This isozyme has glucuronidating capacity with steroid substrates such as 5-beta-androstane 3-alpha,17-beta-diol, estradiol, ADT, eugenol and bile acids. Only isoform 1 seems to be active.
Gene Name:
UGT2B28
Uniprot ID:
Q9BY64
Molecular weight:
38742.9
Reactions
Uridine diphosphate glucuronic acid + Bilirubin → Uridine 5'-diphosphate + Bilirubin diglucuronidedetails
General function:
Involved in transferase activity, transferring hexosyl groups
Specific function:
UDPGTs are of major importance in the conjugation and subsequent elimination of potentially toxic xenobiotics and endogenous compounds. This isozyme is active on polyhydroxylated estrogens (such as estriol, 4-hydroxyestrone and 2-hydroxyestriol) and xenobiotics (such as 4-methylumbelliferone, 1-naphthol, 4-nitrophenol, 2-aminophenol, 4-hydroxybiphenyl and menthol). It is capable of 6 alpha-hydroxyglucuronidation of hyodeoxycholic acid.
Gene Name:
UGT2B4
Uniprot ID:
P06133
Molecular weight:
60512.035
Reactions
Uridine diphosphate glucuronic acid + Bilirubin → Uridine 5'-diphosphate + Bilirubin diglucuronidedetails
General function:
Involved in transferase activity, transferring hexosyl groups
Specific function:
UDPGT is of major importance in the conjugation and subsequent elimination of potentially toxic xenobiotics and endogenous compounds. This isoform glucuronidates bilirubin IX-alpha to form both the IX-alpha-C8 and IX-alpha-C12 monoconjugates and diconjugate.
Gene Name:
UGT1A4
Uniprot ID:
P22310
Molecular weight:
60024.535
Reactions
Uridine diphosphate glucuronic acid + Bilirubin → Uridine 5'-diphosphate + Bilirubin diglucuronidedetails
General function:
Involved in transferase activity, transferring hexosyl groups
Specific function:
UDPGT is of major importance in the conjugation and subsequent elimination of potentially toxic xenobiotics and endogenous compounds.
Gene Name:
UGT2B10
Uniprot ID:
P36537
Molecular weight:
60773.485
Reactions
Uridine diphosphate glucuronic acid + Bilirubin → Uridine 5'-diphosphate + Bilirubin diglucuronidedetails
General function:
Involved in transferase activity, transferring hexosyl groups
Specific function:
UDPGT is of major importance in the conjugation and subsequent elimination of potentially toxic xenobiotics and endogenous compounds. Its unique specificity for 3,4-catechol estrogens and estriol suggests it may play an important role in regulating the level and activity of these potent and active estrogen metabolites. Is also active with androsterone, hyodeoxycholic acid and tetrachlorocatechol (in vitro).
Gene Name:
UGT2B7
Uniprot ID:
P16662
Molecular weight:
60720.15
Reactions
Uridine diphosphate glucuronic acid + Bilirubin → Uridine 5'-diphosphate + Bilirubin diglucuronidedetails
General function:
Involved in transferase activity, transferring hexosyl groups
Specific function:
UDPGTs are of major importance in the conjugation and subsequent elimination of potentially toxic xenobiotics and endogenous compounds. This isozyme displays activity toward several classes of xenobiotic substrates, including simple phenolic compounds, 7-hydroxylated coumarins, flavonoids, anthraquinones, and certain drugs and their hydroxylated metabolites. It also catalyzes the glucuronidation of endogenous estrogens and androgens.
Gene Name:
UGT2B15
Uniprot ID:
P54855
Molecular weight:
61035.815
Reactions
Uridine diphosphate glucuronic acid + Bilirubin → Uridine 5'-diphosphate + Bilirubin diglucuronidedetails
General function:
Involved in transferase activity, transferring hexosyl groups
Specific function:
UDP-glucuronosyltransferases catalyze phase II biotransformation reactions in which lipophilic substrates are conjugated with glucuronic acid to increase water solubility and enhance excretion. They are of major importance in the conjugation and subsequent elimination of potentially toxic xenobiotics and endogenous compounds. Active on odorants and seems to be involved in olfaction; it could help clear lipophilic odorant molecules from the sensory epithelium.
Gene Name:
UGT2A1
Uniprot ID:
Q9Y4X1
Molecular weight:
60771.605
Reactions
Uridine diphosphate glucuronic acid + Bilirubin → Uridine 5'-diphosphate + Bilirubin diglucuronidedetails
General function:
Involved in transferase activity, transferring hexosyl groups
Specific function:
UDPGT is of major importance in the conjugation and subsequent elimination of potentially toxic xenobiotics and endogenous compounds. This isoform glucuronidates bilirubin IX-alpha to form both the IX-alpha-C8 and IX-alpha-C12 monoconjugates and diconjugate. Is also able to catalyze the glucuronidation of 17beta-estradiol, 17alpha-ethinylestradiol, 1-hydroxypyrene, 4-methylumbelliferone, 1-naphthol, paranitrophenol, scopoletin, and umbelliferone.
Gene Name:
UGT1A1
Uniprot ID:
P22309
Molecular weight:
59590.91
Reactions
Uridine diphosphate glucuronic acid + Bilirubin → Uridine 5'-diphosphate + Bilirubin diglucuronidedetails
General function:
Involved in transferase activity, transferring hexosyl groups
Specific function:
UDPGT is of major importance in the conjugation and subsequent elimination of potentially toxic xenobiotics and endogenous compounds. This isoform has specificity for phenols.
Gene Name:
UGT1A9
Uniprot ID:
O60656
Molecular weight:
59940.495
Reactions
Uridine diphosphate glucuronic acid + Bilirubin → Uridine 5'-diphosphate + Bilirubin diglucuronidedetails

Transporters

General function:
Involved in transporter activity
Specific function:
Mediates the Na(+)-independent transport of organic anions such as pravastatin, taurocholate, methotrexate, dehydroepiandrosterone sulfate, 17-beta-glucuronosyl estradiol, estrone sulfate, prostaglandin E2, thromboxane B2, leukotriene C3, leukotriene E4, thyroxine and triiodothyronine. May play an important role in the clearance of bile acids and organic anions from the liver
Gene Name:
SLCO1B1
Uniprot ID:
Q9Y6L6
Molecular weight:
76448.0
References
  1. Zhang W, He YJ, Gan Z, Fan L, Li Q, Wang A, Liu ZQ, Deng S, Huang YF, Xu LY, Zhou HH: OATP1B1 polymorphism is a major determinant of serum bilirubin level but not associated with rifampicin-mediated bilirubin elevation. Clin Exp Pharmacol Physiol. 2007 Dec;34(12):1240-4. [PubMed:17973861 ]
General function:
Involved in ATP binding
Specific function:
Mediates hepatobiliary excretion of numerous organic anions. May function as a cellular cisplatin transporter
Gene Name:
ABCC2
Uniprot ID:
Q92887
Molecular weight:
174205.6
References
  1. Watanabe N, Takashimizu S, Kojima S, Kagawa T, Nishizaki Y, Mine T, Matsuzaki S: Clinical and pathological features of a prolonged type of acute intrahepatic cholestasis. Hepatol Res. 2007 Aug;37(8):598-607. Epub 2007 May 22. [PubMed:17517076 ]

Only showing the first 10 proteins. There are 28 proteins in total.