You are using an unsupported browser. Please upgrade your browser to a newer version to get the best experience on Human Metabolome Database.
Record Information
Version4.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2019-01-11 19:14:52 UTC
HMDB IDHMDB0000643
Secondary Accession Numbers
  • HMDB00643
Metabolite Identification
Common NameCoproporphyrin I
DescriptionCoproporphyrin I is a porphyrin metabolite arising from heme synthesis. Porphyrins are pigments found in both animal and plant life. Coproporphyrin I is a tetrapyrrole dead-end product from the spontaneous oxidation of the methylene bridges of coproporphynogen, arising from heme synthesis and secreted in feces and urine. Increased levels of coproporphyrins can indicate congenital erythropoietic porphyria or sideroblastic anaemia. Porphyria is a pathological state characterised 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, 3) the toxic form due to occupational and environmental exposure takes mainly a subclinical course. Hepatic factors includes disturbance in coproporphyrinogen metabolism, which results from inhibition of coproporphyrinogen oxidase as well as from the rapid loss from, and diminished utilization of coproporphyrinogen in the hepatocytes, which may also explain why coproporphyrin, its autoxidation product, predominates physiologically in the urine; decreased biliary excretion of coproporphyrin leading to a compensatory urinary excretion, so that the coproporphyrin ring isomer ratio (1:III) becomes a sensitive index for impaired liver function and 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 ).
Structure
Data?1547234092
SynonymsNot Available
Chemical FormulaC36H38N4O8
Average Molecular Weight654.7089
Monoisotopic Molecular Weight654.268964212
IUPAC NameNot Available
Traditional NameNot Available
CAS Registry Number531-14-6
SMILESNot Available
InChI Identifier
InChI=1S/C36H38N4O8/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/h13-16,37,40H,5-12H2,1-4H3,(H,41,42)(H,43,44)(H,45,46)(H,47,48)/b25-13-,26-14-,27-15-,28-16-,29-14-,30-13-,31-16-,32-15-
InChI KeyVORBHEGMEBOMMB-JRHDEHKPSA-N
Chemical Taxonomy
ClassificationNot classified
Ontology
Physiological effect

Health effect:

Disposition

Source:

Biological location:

Process

Naturally occurring process:

Role

Industrial application:

Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted PropertiesNot Available
Spectra
Spectrum TypeDescriptionSplash KeyView
Biological Properties
Cellular Locations
  • Cytoplasm
  • Mitochondria
Biospecimen Locations
  • Blood
  • Urine
Tissue Locations
  • Liver
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.0073 +/- 0.0035 uMNewborn (0-30 days old)BothNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified0.012 +/- 0.005 uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified0.005 (0.000 - 0.010) uMAdult (>18 years old)BothNormal details
UrineDetected and Quantified0.0021 +/- 0.001 umol/mmol creatinineAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified0.0020 +/- 0.0011 umol/mmol creatinineAdult (>18 years old)MaleNormal details
UrineDetected and Quantified0.0049 +/- 0.00019 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified0.0036 +/- 0.0015 umol/mmol creatinineAdult (>18 years old)BothNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
details
UrineDetected and Quantified0.0098 (0.0053-0.017) umol/mmol creatinineNewborn (0-30 days old)BothNormal details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.008 (0.000 - 0.016) uMAdult (>18 years old)Both
Porphyria
details
BloodDetected and Quantified0.0850 (0.0008 - 0.1700) uMAdult (>18 years old)MalePorphyria details
BloodDetected and Quantified0 uMAdult (>18 years old)MaleProtoporphyria, Erythropoietic details
UrineDetected and Quantified0.0042 +/- 0.00019 umol/mmol creatinineAdult (>18 years old)BothLiver disease details
UrineDetected and Quantified0.002 +/- 0.00019 umol/mmol creatinineAdult (>18 years old)BothPorphyria cutanea tarda details
UrineDetected and Quantified0.014 +/- 0.00013 umol/mmol creatinineAdult (>18 years old)BothPorphyria variegata details
UrineDetected and Quantified0.0019 +/- 0.00085 umol/mmol creatinineAdult (>18 years old)BothHereditary coproporphyria details
UrineDetected and Quantified0.0035 +/- 0.00013 umol/mmol creatinineAdult (>18 years old)BothAcute intermittent porphyria details
UrineDetected and Quantified0.015 (0.0067-0.021) umol/mmol creatinineNot SpecifiedBothHexachlorobenzene exposure details
UrineDetected and Quantified0.00326-0.00916 umol/mmol creatinineAdult (>18 years old)MaleProtoporphyria, Erythropoietic details
Associated Disorders and Diseases
Disease References
Porphyria
  1. Hindmarsh JT, Oliveras L, Greenway DC: Biochemical differentiation of the porphyrias. Clin Biochem. 1999 Nov;32(8):609-19. [PubMed:10638943 ]
Protoporphyria, Erythropoietic
  1. MAGNUS IA, JARRETT A, PRANKERD TA, RIMINGTON C: Erythropoietic protoporphyria. A new porphyria syndrome with solar urticaria due to protoporphyrinaemia. Lancet. 1961 Aug 26;2(7200):448-51. [PubMed:13765301 ]
Liver disease
  1. Zuijderhoudt FM, Koehorst SG, Kluitenberg WE, Dorresteijn-de Bok J: On accuracy and precision of a HPLC method for measurement of urine porphyrin concentrations. Clin Chem Lab Med. 2000 Mar;38(3):227-30. [PubMed:10905759 ]
Porphyria cutanea tarda
  1. Zuijderhoudt FM, Koehorst SG, Kluitenberg WE, Dorresteijn-de Bok J: On accuracy and precision of a HPLC method for measurement of urine porphyrin concentrations. Clin Chem Lab Med. 2000 Mar;38(3):227-30. [PubMed:10905759 ]
Variegate porphyria
  1. Zuijderhoudt FM, Koehorst SG, Kluitenberg WE, Dorresteijn-de Bok J: On accuracy and precision of a HPLC method for measurement of urine porphyrin concentrations. Clin Chem Lab Med. 2000 Mar;38(3):227-30. [PubMed:10905759 ]
Hereditary coproporphyria
  1. Zuijderhoudt FM, Koehorst SG, Kluitenberg WE, Dorresteijn-de Bok J: On accuracy and precision of a HPLC method for measurement of urine porphyrin concentrations. Clin Chem Lab Med. 2000 Mar;38(3):227-30. [PubMed:10905759 ]
Acute intermittent porphyria
  1. Zuijderhoudt FM, Koehorst SG, Kluitenberg WE, Dorresteijn-de Bok J: On accuracy and precision of a HPLC method for measurement of urine porphyrin concentrations. Clin Chem Lab Med. 2000 Mar;38(3):227-30. [PubMed:10905759 ]
Associated OMIM IDs
  • 177000 (Protoporphyria, Erythropoietic)
  • 176200 (Variegate porphyria)
  • 121300 (Hereditary coproporphyria)
  • 176000 (Acute intermittent porphyria)
External LinksNot Available
References
Synthesis ReferenceSchonheyder, Fritz. The formation of coproporphyrin I and hemoglobin during embryonic life. Journal of Biological Chemistry (1938), 123 491-7.
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. Doss MO: Porphyrinurias and occupational disease. Ann N Y Acad Sci. 1987;514:204-18. [PubMed:3327428 ]