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Record Information
Version4.0
StatusDetected and Quantified
Creation Date2006-05-22 14:17:39 UTC
Update Date2018-03-04 06:57:11 UTC
HMDB IDHMDB0002158
Secondary Accession Numbers
  • HMDB02158
Metabolite Identification
Common NameCoproporphyrinogen I
DescriptionCoproporphyrinogen I is a porphyrin metabolite arising from heme synthesis. Porphyrins are pigments found in both animal and plant life. Coproporphyrinogen I is a tetrapyrrole dead-end product resulting from the spontaneous oxidation of the methylene bridges of coproporphyrinogen arising from heme synthesis. It is secreted in feces and urine. Coproporphyrinogen I is biosynthesized from the tetrapyrrole hydroxymethylbilane, which is converted by the action of uroporphyrinogen synthase to uroporphyrinogen I. Uroporphyrinogen I is subsequently converted into coproporphyrinogen I through a series of four decarboxylations. Increased levels of coproporphyrinogens can indicate congenital erythropoietic porphyria or sideroblastic anemia, which are inherited disorders. Porphyria is a pathological state characterized by abnormalities of porphyrin metabolism and results in the excretion of large quantities of porphyrins in the urine and in extreme sensitivity to light. A large number of factors are capable of increasing porphyrin excretion, owing to different and multiple causes and etiologies: (1) the main site of the chronic hepatic porphyria disease process concentrates on the liver, (2) a functional and morphologic liver injury is almost regularly associated with this chronic porphyria, and (3) the toxic form due to occupational and environmental exposure takes mainly a subclinical course. Hepatic factors include disturbance in coproporphyrinogen metabolism, which results from inhibition of coproporphyrinogen oxidase as well as from the rapid loss and diminished utilization of coproporphyrinogen in the hepatocytes. This may also explain why coproporphyrin, its autoxidation product, predominates physiologically in the urine. Decreased biliary excretion of coproporphyrin leading to a compensatory urinary excretion. Therefore, the coproporphyrin ring isomer ratio becomes a sensitive index for impaired liver function, intrahepatic cholestasis, and disturbed activity of hepatic uroporphyrinogen decarboxylase. In itself, secondary coproporphyrinuria is not associated with porphyria symptoms of a hepatologic-gastroenterologic, neurologic, or dermatologic order, even though coproporphyrinuria can occur with such symptoms (PMID: 3327428 ). Under certain conditions, coproporphyrinogen I can act as a phototoxin, a neurotoxin, and a metabotoxin. A phototoxin leads to cell damage upon exposure to light. A neurotoxin causes damage to nerve cells and nerve tissues. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of porphyrins are associated with porphyrias such as porphyria variegate, acute intermittent porphyria, hereditary coproporphyria (HCP), congenital erythropoietic porphyria, and sideroblastic anemia. There are several types of porphyrias (most are inherited). Hepatic porphyrias are characterized by acute neurological attacks (seizures, psychosis, extreme back and abdominal pain, and an acute polyneuropathy), while the erythropoietic forms present with skin problems (usually a light-sensitive blistering rash and increased hair growth). The neurotoxicity of porphyrins may be due to their selective interactions with tubulin, which disrupt microtubule formation and cause neural malformations (PMID: 3441503 ). Coproporphyrinogen I can be found in a number of food items, including cascade huckleberry, hyacinth bean, horseradish tree, and watercress.
Structure
Thumb
Synonyms
ValueSource
3,8,13,18-Tetramethyl-5,10,15,20,22,24-hexahydroporphyrin-2,7,12,17-tetrapropionic acidChEBI
COPROPORPHYRIN IChEBI
3,8,13,18-Tetramethyl-5,10,15,20,22,24-hexahydroporphyrin-2,7,12,17-tetrapropionateGenerator
3,8,13,18-Tetramethyl-5,10,15,20,22,24-hexahydroporphyrin-2,7,12,17-tetrapropanoateHMDB
3,8,13,18-Tetramethyl-5,10,15,20,22,24-hexahydroporphyrin-2,7,12,17-tetrapropanoic acidHMDB
Chemical FormulaC36H44N4O8
Average Molecular Weight660.7566
Monoisotopic Molecular Weight660.315914404
IUPAC Name3-[9,14,19-tris(2-carboxyethyl)-5,10,15,20-tetramethyl-21,22,23,24-tetraazapentacyclo[16.2.1.1³,⁶.1⁸,¹¹.1¹³,¹⁶]tetracosa-1(20),3,5,8,10,13,15,18-octaen-4-yl]propanoic acid
Traditional Namecoproporphyrinogen I
CAS Registry Number31110-56-2
SMILES
CC1=C2CC3=C(CCC(O)=O)C(C)=C(CC4=C(CCC(O)=O)C(C)=C(CC5=C(CCC(O)=O)C(C)=C(CC(N2)=C1CCC(O)=O)N5)N4)N3
InChI Identifier
InChI=1S/C36H44N4O8/c1-17-21(5-9-33(41)42)29-14-26-19(3)23(7-11-35(45)46)31(39-26)16-28-20(4)24(8-12-36(47)48)32(40-28)15-27-18(2)22(6-10-34(43)44)30(38-27)13-25(17)37-29/h37-40H,5-16H2,1-4H3,(H,41,42)(H,43,44)(H,45,46)(H,47,48)
InChI KeyWIUGGJKHYQIGNH-UHFFFAOYSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as porphyrins. These are compounds containing a fundamental skeleton of four pyrrole nuclei united through the alpha-positions by four methine groups to form a macrocyclic structure.
KingdomOrganic compounds
Super ClassOrganoheterocyclic compounds
ClassTetrapyrroles and derivatives
Sub ClassPorphyrins
Direct ParentPorphyrins
Alternative Parents
Substituents
  • Porphyrin
  • Tetracarboxylic acid or derivatives
  • Substituted pyrrole
  • Pyrrole
  • Heteroaromatic compound
  • Carboxylic acid derivative
  • Carboxylic acid
  • Azacycle
  • Carbonyl group
  • Hydrocarbon derivative
  • Organic oxide
  • Organopnictogen compound
  • Organooxygen compound
  • Organonitrogen compound
  • Organic oxygen compound
  • Organic nitrogen compound
  • Aromatic heteropolycyclic compound
Molecular FrameworkAromatic heteropolycyclic compounds
External Descriptors
Ontology
Physiological effect

Health effect:

Disposition

Route of exposure:

Source:

Biological location:

Process

Naturally occurring process:

Role

Indirect biological role:

Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point171 - 174 °CNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility0.011 g/LALOGPS
logP1.97ALOGPS
logP4.89ChemAxon
logS-4.8ALOGPS
pKa (Strongest Acidic)3.85ChemAxon
Physiological Charge-4ChemAxon
Hydrogen Acceptor Count8ChemAxon
Hydrogen Donor Count8ChemAxon
Polar Surface Area212.36 ŲChemAxon
Rotatable Bond Count12ChemAxon
Refractivity181.86 m³·mol⁻¹ChemAxon
Polarizability71.34 ųChemAxon
Number of Rings5ChemAxon
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, Positivesplash10-0w2c-2000019000-995b0bc8ddfaee513a88View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-01ox-0000029000-7cfed66d72503c62d69fView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-07c7-0000098000-c52b6ccc51ad35ceecd9View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-0a4i-0102190000-338272475aad39d99e29View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-0a4i-0000009000-68fb68abaac4161fb473View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-0a4i-1000029000-03bf0dcb10bc9f0283a9View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0a4l-9000043000-4a0f162250fd839a83b3View in MoNA
Biological Properties
Cellular Locations
  • Membrane (predicted from logP)
Biospecimen Locations
  • Blood
Tissue Location
  • Spleen
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 Quantified0.015 (0.01 - 0.02) uMAdult (>18 years old)Not SpecifiedNormal details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.5115 (0.3875 - 0.6355) uMAdult (>18 years old)Male
Porphyria
details
Associated Disorders and Diseases
Disease References
Porphyria
  1. Gorchein A, Guo R, Lim CK, Raimundo A, Pullon HW, Bellingham AJ: Porphyrins in urine, plasma, erythrocytes, bile and faeces in a case of congenital erythropoietic porphyria (Gunther's disease) treated with blood transfusion and iron chelation: lack of benefit from oral charcoal. Biomed Chromatogr. 1998 Nov-Dec;12(6):350-6. [PubMed:9861496 ]
Associated OMIM IDsNone
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FoodDB IDFDB022874
KNApSAcK IDNot Available
Chemspider ID389645
KEGG Compound IDC05768
BioCyc IDCOPROPORPHYRINOGEN_I
BiGG IDNot Available
Wikipedia LinkNot Available
METLIN ID6516
PubChem Compound440776
PDB ID1CP
ChEBI ID28607
References
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Ding Y, Lin B, Huie CW: Binding studies of porphyrins to human serum albumin using affinity capillary electrophoresis. Electrophoresis. 2001 Jul;22(11):2210-6. [PubMed:11504054 ]
  2. Gorchein A, Guo R, Lim CK, Raimundo A, Pullon HW, Bellingham AJ: Porphyrins in urine, plasma, erythrocytes, bile and faeces in a case of congenital erythropoietic porphyria (Gunther's disease) treated with blood transfusion and iron chelation: lack of benefit from oral charcoal. Biomed Chromatogr. 1998 Nov-Dec;12(6):350-6. [PubMed:9861496 ]
  3. Beukeveld GJ, In 't Veld G, Havinga R, Groen AK, Wolthers BG, Kuipers F: Relationship between biliary lipid and protoporphyrin secretion; potential role of mdr2 P-glycoprotein in hepatobiliary organic anion transport. J Hepatol. 1996 Mar;24(3):343-52. [PubMed:8778203 ]
  4. Pinelli A, Mussini C, Bertolini B, Buratti M, Trivulzio S: Increased excretion of urine coproporphyrins during daunorubicin administration in patients affected by acute myelogenous leukemia. Pharmacol Res. 2003 Nov;48(5):515-8. [PubMed:12967599 ]
  5. Sakai T, Niinuma Y, Yanagihara S, Ushio K: Liquid-chromatographic separation and determination of coproporphyrins I and III in urine. Clin Chem. 1983 Feb;29(2):350-3. [PubMed:6821943 ]
  6. Cornford P: Transformation of porphobilinogen into porphyrins by preparations from human erythrocytes. Biochem J. 1964 Apr;91(1):64-73. [PubMed:5833390 ]
  7. Pannier E, Viot G, Aubry MC, Grange G, Tantau J, Fallet-Bianco C, Muller F, Cabrol D: Congenital erythropoietic porphyria (Gunther's disease): two cases with very early prenatal manifestation and cystic hygroma. Prenat Diagn. 2003 Jan;23(1):25-30. [PubMed:12533808 ]
  8. Mel'nikova YaI, Kravchuk ZI, Preygerzon VA, Martsev SP: Functional activation of antibodies on modification with Pd(II) coproporphyrin I N-Hydroxysuccinimide ester. Biochemistry (Mosc). 1997 Aug;62(8):924-7. [PubMed:9360305 ]
  9. Doss MO: Porphyrinurias and occupational disease. Ann N Y Acad Sci. 1987;514:204-18. [PubMed:3327428 ]
  10. Winkelman JW, Collins GH: Neurotoxicity of tetraphenylporphinesulfonate TPPS4 and its relation to photodynamic therapy. Photochem Photobiol. 1987 Nov;46(5):801-7. [PubMed:3441503 ]

Enzymes

General function:
Involved in uroporphyrinogen decarboxylase activity
Specific function:
Catalyzes the decarboxylation of four acetate groups of uroporphyrinogen-III to yield coproporphyrinogen-III.
Gene Name:
UROD
Uniprot ID:
P06132
Molecular weight:
40786.58
Reactions
Uroporphyrinogen I → Coproporphyrinogen I + Carbon dioxidedetails