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Human Metabolome Database Version 2.5

 

Showing metabocard for Coproporphyrin III (HMDB00570)

Legend: metabolite field enzyme field

Version 2.5
Creation Date 2005-11-16 15:48:42
Update Date 2009-09-10 09:55:28
Accession Number HMDB00570
Secondary Accession Numbers Not Available
Common Name Coproporphyrin III
Description Coproporhyrin III is a porphyrin metabolite arising from heme synthesis. Porphyrins are pigments found in both animal and plant life. Coproporphyrin III 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)
Synonyms Not Available
Chemical IUPAC Name 3,8,13,17-tetramethylporphyrin-2,7,12,18-tetrapropanoic acid
Chemical Formula C36H38N4O8
Chemical Structure Structure
Chemical Taxonomy
Kingdom
  • Organic
Super Class
  • Heterocyclic molecules
Class
  • Porphyrins
Sub Class
  • Miscellaneous porphyrins
Family
  • Mammalian Metabolite
Species
  • carboxylic acid
  • aromatic compound
  • heterocyclic compound
Biofunction
—
Application
—
Source
  • Endogenous
Average Molecular Weight 654.709
Monoisotopic Molecular Weight 654.268982
Isomeric SMILES CC1=C(CCC(O)=O)/C2=C/C3=N/C(=CC4=C(C)C(CCC(O)=O)=C(N4)C=C4/N=C(/C=C1N2)C(C)=C/4CCC(O)=O)/C(CCC(O)=O)=C3C
Canonical SMILES CC1=C(CCC(O)=O)C2=CC3=NC(=CC4=C(C)C(CCC(O)=O)=C(N4)C=C4N=C(C=C1N2)C(C)=C4CCC(O)=O)C(CCC(O)=O)=C3C
KEGG Compound ID C03263 Link Image
BioCyc ID Not Available
BiGG ID Not Available
Wikipedia Link Not Available
NuGOwiki Link HMDB00570 Link Image
Metagene Link HMDB00570 Link Image
METLIN ID 5553 Link Image
PubChem Compound 114935 Link Image
PubChem Substance 691877 Link Image
ChEBI ID 27609 Link Image
CAS Registry Number 14643-66-4
InChI Identifier InChI=1/C36H38N4O8/c1-17-21(5-9-33(41)42)29-14-27-19(3)22(6-10-34(43)44)30(39-27)15-28-20(4)24(8-12-36(47)48)32(40-28)16-31-23(7-11-35(45)46)18(2)26(38-31)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-,26-13-,27-14-,28-15-,29-14-,30-15-,31-16-,32-16-
Synthesis Reference Minoda, Taiji; Takada, Toshihiro; Horii, Shinichi. Coproporphyrin III. Jpn. Kokai Tokkyo Koho (1979), 3 pp.
Melting Point (Experimental) Not Available
Experimental Water Solubility Not Available Source: PhysProp
Predicted Water Solubility 0.0307 mg/mL [Predicted by ALOGPS] Calculated using ALOGPS
Physiological Charge -4
State Solid
Experimental LogP/Hydrophobicity Not Available Source: PhysProp
Predicted LogP/Hydrophobicity 2.53 [Predicted by ALOGPS]; 0.7 [Predicted by PubChem via XLOGP] Calculated using ALOGPS
Material Safety Data Sheet (MSDS) Not Available
MOL File Show
SDF File Show
PDB File Show
2D Structure
3D Structure
Experimental PDB ID 1R3T Link Image
Experimental PDB File Show
Experimental PDB Structure
Experimental 1H NMR Spectrum Not Available
Experimental 13C NMR Spectrum Not Available
Experimental 13C HSQC Spectrum Not Available
Predicted 1H NMR Spectrum Show Image
Show Peaklist
Predicted 13C NMR Spectrum Show Image
Show Peaklist
Mass Spectrum Not Available
Simplified TOCSY Spectrum Not Available
BMRB Spectrum Not Available
Cellular Location
  • Cytoplasm
  • Mitochondria
Biofluid Location
  • Blood
  • Urine
Tissue Location
Tissue References
Liver —
Concentrations (Normal)
Biofluid Blood
Value 0.006 (0.000 - 0.012) uM
Age Adult:>18 yrs old
Sex N/A
Patient information Normal
Comments Not Available
References
  • Hindmarsh JT, Oliveras L, Greenway DC: Biochemical differentiation of the porphyrias. Clin Biochem. 1999 Nov;32(8):609-19. [PubMed Link Image]
Biofluid Urine
Value 0.0051 +/- 0.0014 umol/mmol creatinine
Age Adult:>18 yrs old
Sex Male
Patient information Normal
Comments Not Available
References
  • Li J, Cai Z: Stacking and separation of urinary porphyrins in capillary electrophoresis: optimization of concentration efficiency and resolution. Talanta. 2008 Oct 19;77(1):331-9. Epub 2008 Jul 1. [PubMed Link Image]
Biofluid Urine
Value 0.0094 +/- 0.00013 umol/mmol creatinine
Age Adult:>18 yrs old
Sex Both
Patient information Normal
Comments Not Available
References
  • 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 Link Image]
Concentrations (Abnormal)
Biofluid Blood
Value 0.0045 (0.0000 - 0.0090) uM
Age Adult:>18 yrs old
Sex N/A
Condition Porphyria
Comments Hereditary Coproporphyria
References
  • Hindmarsh JT, Oliveras L, Greenway DC: Biochemical differentiation of the porphyrias. Clin Biochem. 1999 Nov;32(8):609-19. [PubMed Link Image]
Biofluid Blood
Value 0.0044 (0.0002 - 0.0087) uM
Age Adult:>18 yrs old
Sex N/A
Condition Porphyria
Comments Porphyria Variegata
References
  • Hindmarsh JT, Oliveras L, Greenway DC: Biochemical differentiation of the porphyrias. Clin Biochem. 1999 Nov;32(8):609-19. [PubMed Link Image]
Biofluid Urine
Value 0.0054 +/- 0.0019 umol/mmol creatinine
Age Adult:>18 yrs old
Sex Female
Condition Abnormal
Comments Not Available
References
  • Li J, Cai Z: Stacking and separation of urinary porphyrins in capillary electrophoresis: optimization of concentration efficiency and resolution. Talanta. 2008 Oct 19;77(1):331-9. Epub 2008 Jul 1. [PubMed Link Image]
Biofluid Urine
Value 0.012 +/- 0.00013 umol/mmol creatinine
Age Adult:>18 yrs old
Sex Both
Condition Liver disease
Comments Not Available
References
  • 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 Link Image]
Biofluid Urine
Value 0.005 umol/mmol creatinine
Age Adult:>18 yrs old
Sex Both
Comments Not Available
References
  • 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 Link Image]
Biofluid Urine
Value 0.015 +/- 0.00078 umol/mmol creatinine
Age Adult:>18 yrs old
Sex Both
Condition Hereditary coproporphyria
Comments Not Available
References
  • 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 Link Image]
Biofluid Urine
Value 0.043 +/- 0.00019 umol/mmol creatinine
Age Adult:>18 yrs old
Sex Both
Comments Not Available
References
  • 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 Link Image]
Associated Disorders
Condition References
Hereditary coproporphyria
  • 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 Link Image]
Liver disease
  • 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 Link Image]
Porphyria
  • Hindmarsh JT, Oliveras L, Greenway DC: Biochemical differentiation of the porphyrias. Clin Biochem. 1999 Nov;32(8):609-19. [PubMed Link Image]
OMIM ID
Pathways
Name SMPDB Link KEGG Link
Porphyrin Metabolism SMP00024 Link Image map00860 Link Image
General References Not Available
Metabolic Enzymes
  1. cDNA FLJ76900, highly similar to Homo sapiens uroporphyrinogen decarboxylase
  2. cDNA FLJ76234, highly similar to Homo sapiens coproporphyrinogen oxidase
Enzyme 1 [top]
Enzyme 1 ID 13084
Enzyme 1 Name cDNA FLJ76900, highly similar to Homo sapiens uroporphyrinogen decarboxylase
Enzyme 1 Synonyms
  1. UROD, mRNA
  2. Uroporphyrinogen decarboxylase, isoform CRA_a
Enzyme 1 Gene Name UROD
Enzyme 1 Protein Sequence >cDNA FLJ76900, highly similar to Homo sapiens uroporphyrinogen decarboxylase 
MEANGLGPQGFPELKNDTFLRAAWGEETDYTPVWCMRQAGRYLPEFRETRAAQDFFSTCR
SPEACCELTLQPLRRFPLDAAIIFSDILVVPQALGMEVTMVPGKGPSFPEPLREEQDLER
LRDPEVVASELGYVFQAITLTRQRLAGRVPLIGFAGAPWTLMTYMVEGGGSSTMAQAKRW
LYQRPQASHQLLRILTDALVPYLVGQVVAGAQALQLFESHAGHLGPQLFNKFALPYIRDV
AKQVKARLREAGLAPVPMIIFAKDGHFALEELAQAGYEVVGLDWTVAPKKARECVGKTVT
LQGNLDPCALYASEEEIGQLVKQMLDDFGPHRYIANLGHGLYPDMDPEHVGAFVDAVHKH
SRLLRQN
Enzyme 1 Number of Residues 367
Enzyme 1 Molecular Weight 40787
Enzyme 1 Theoretical pI 6.06
Enzyme 1 GO Classification Not Available
Enzyme 1 General Function Coenzyme transport and metabolism
Enzyme 1 Specific Function Not Available
Enzyme 1 Pathways Not Available
Enzyme 1 Reactions Not Available
Enzyme 1 Pfam Domain Function Not Available
Enzyme 1 Signals
  • None
Enzyme 1 Transmembrane Regions
  • None
Enzyme 1 Essentiality Not Available
Enzyme 1 GenBank ID Protein 158257186 Link Image
Enzyme 1 UniProtKB/Swiss-Prot ID A8K762 Link Image
Enzyme 1 UniProtKB/Swiss-Prot Entry Name A8K762_HUMAN Link Image
Enzyme 1 PDB ID 1R3Y Link Image
Enzyme 1 PDB File Show
Enzyme 1 3D Structure
Enzyme 1 Cellular Location Not Available
Enzyme 1 Gene Sequence Not Available
Enzyme 1 GenBank Gene ID AK291877 Link Image
Enzyme 1 GeneCard ID A8K762 Link Image
Enzyme 1 GenAtlas ID Not Available
Enzyme 1 HGNC ID Not Available
Enzyme 1 Chromosome Location Not Available
Enzyme 1 Locus Not Available
Enzyme 1 SNPs SNPJam Report Link Image
Enzyme 1 General References Not Available
Enzyme 1 Metabolite References Not Available
Enzyme 2 [top]
Enzyme 2 ID 13085
Enzyme 2 Name cDNA FLJ76234, highly similar to Homo sapiens coproporphyrinogen oxidase
Enzyme 2 Synonyms
  1. CPOX, mRNA
  2. Coproporphyrinogen oxidase
Enzyme 2 Gene Name CPOX
Enzyme 2 Protein Sequence >cDNA FLJ76234, highly similar to Homo sapiens coproporphyrinogen oxidase 
MALQLGRLSSGPCWLVARGGCGGPRAWSQCGGGGLRAWSQRSAAGRVCRPPGPAGTEQSR
GLGHGSTSRGGPWVGTGLAAALAGLVGLATAAFGHVQRAEMLPKTSGTRATSLGRPEEEE
DELAHRCSSFMAPPVTDLGELRRRPGDMKTKMELLILETQAQVCQALAQVDGGANFSVDR
WERKEGGGGISCVLQDGCVFEKAGVSISVVHGNLSEEAAKQMRSRGKVLKTKDGKLPFCA
MGVSSVIHPKNPHAPTIHFNYRYFEVEEADGNKQWWFGGGCDLTPTYLNQEDAVHFHRTL
KEACDQHGPDLYPKFKKWCDDYFFIAHRGERRGIGGIFFDDLDSPSKEEVFRFVQSCARA
VVPSYIPLVKKHCDDSFTPQEKLWQQLRRGRYVEFNLLYDRGTKFGLFTPGSRIESILMS
LPLTARWEYMHSPSENSKEAEILEVLRHPRDWVR
Enzyme 2 Number of Residues 454
Enzyme 2 Molecular Weight 50152
Enzyme 2 Theoretical pI 8.33
Enzyme 2 GO Classification Not Available
Enzyme 2 General Function Coenzyme transport and metabolism
Enzyme 2 Specific Function Not Available
Enzyme 2 Pathways Not Available
Enzyme 2 Reactions Not Available
Enzyme 2 Pfam Domain Function Not Available
Enzyme 2 Signals
  • None
Enzyme 2 Transmembrane Regions
  • None
Enzyme 2 Essentiality Not Available
Enzyme 2 GenBank ID Protein 158261303 Link Image
Enzyme 2 UniProtKB/Swiss-Prot ID A8K275 Link Image
Enzyme 2 UniProtKB/Swiss-Prot Entry Name A8K275_HUMAN Link Image
Enzyme 2 PDB ID Not Available
Enzyme 2 Cellular Location Not Available
Enzyme 2 Gene Sequence Not Available
Enzyme 2 GenBank Gene ID AK290140 Link Image
Enzyme 2 GeneCard ID A8K275 Link Image
Enzyme 2 GenAtlas ID Not Available
Enzyme 2 HGNC ID Not Available
Enzyme 2 Chromosome Location Not Available
Enzyme 2 Locus Not Available
Enzyme 2 SNPs SNPJam Report Link Image
Enzyme 2 General References Not Available
Enzyme 2 Metabolite References Not Available