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Record Information
StatusExpected but not Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2018-03-02 22:53:31 UTC
Secondary Accession Numbers
  • HMDB01097
Metabolite Identification
Common NameProtoporphyrinogen IX
DescriptionProtoporphyrinogen IX is an intermediate in heme biosynthesis. It is a porphyrinogen in which two pyrrole rings each have one methyl and one propionate side chain, and the other two pyrrole rings each have one methyl and one vinyl side chain. Fifteen isomers are possible but only one, type IX, occurs naturally. Protoporphyrinogen is produced by oxidative decarboxylation of coproporphyrinogen. Under certain conditions, protoporphyrinogen IX 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, and hereditary coproporphyria (HCP). In particular, protoporphyrinogen IX is accumulated and excreted excessively in the feces in acute intermittent porphyria, protoporphyria, and variegate porphyria. 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 ).
7,12-Diethenyl-3,8,13,17-tetramethyl-5,10,15,20,22,24-hexahydroporphyrin-2,18-dipropanoic acidChEBI
5,10,15,20,22,24-hexahydro Protoporphyrin IX deriv.HMDB
7,12-Diethenyl-5,10,15,20,22,24-hexahydro-3,8,13,17-tetramethyl-21H,23H-porphine-2,18-dipropanoic acidHMDB
Chemical FormulaC34H40N4O4
Average Molecular Weight568.7058
Monoisotopic Molecular Weight568.304955788
IUPAC NameNot Available
Traditional NameNot Available
CAS Registry Number7412-77-3
InChI Identifier
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
  • Porphyrin
  • Dicarboxylic acid or derivatives
  • Substituted pyrrole
  • Pyrrole
  • Heteroaromatic compound
  • Azacycle
  • Carboxylic acid
  • Carboxylic acid derivative
  • Carbonyl group
  • Organopnictogen compound
  • Organic oxygen compound
  • Organooxygen compound
  • Organonitrogen compound
  • Organic nitrogen compound
  • Hydrocarbon derivative
  • Organic oxide
  • Aromatic heteropolycyclic compound
Molecular FrameworkAromatic heteropolycyclic compounds
External Descriptors

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  Tissue and substructures:

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Naturally occurring process:

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    Biochemical pathway:

Physiological effect

Health effect:

  Health condition:

Physical Properties
Experimental Properties
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
Water Solubility0.0055 g/LALOGPS
Spectrum TypeDescriptionSplash Key
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-0pi3-1000190000-2785151d048ffcfdafc1View in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (1 TMS) - 70eV, Positivesplash10-00bc-9000053000-45009d4bbcc7a57d2e8bView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-0udi-0000190000-7c4ce83e3be73bfccdf6View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-0pi0-0000490000-affa2d20d85ce7a5a2a1View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-03dj-0020900000-a1714c4d96457c66da57View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-014i-0000090000-4148fa37b5b91e3b5a92View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-05tb-1000090000-27a9f5ba745bc29e2b22View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0a4i-9000260000-392765703d0911aa98fcView in MoNA
Biological Properties
Cellular Locations
  • Cytoplasm
  • Membrane (predicted from logP)
  • Mitochondria
Biofluid LocationsNot Available
Tissue Location
  • Liver
  • Placenta
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
Not Available
Abnormal Concentrations
Not Available
Associated Disorders and Diseases
Disease ReferencesNone
Associated OMIM IDsNone
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FoodDB IDFDB022425
KNApSAcK IDC00007371
Chemspider ID108741
KEGG Compound IDC01079
BiGG ID36764
Wikipedia LinkNot Available
PubChem Compound121893
PDB IDNot Available
ChEBI ID15435
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Wan J, Zhang L, Yang G, Zhan CG: Quantitative structure-activity relationship for cyclic imide derivatives of protoporphyrinogen oxidase inhibitors: a study of quantum chemical descriptors from density functional theory. J Chem Inf Comput Sci. 2004 Nov-Dec;44(6):2099-105. [PubMed:15554680 ]
  2. Herrick AL, Moore MR, Thompson GG, Ford GP, McColl KE: Cholelithiasis in patients with variegate porphyria. J Hepatol. 1991 Jan;12(1):50-3. [PubMed:2007776 ]
  3. Krijt J, Stranska P, Maruna P, Vokurka M, Sanitrak J: Herbicide-induced experimental variegate porphyria in mice: tissue porphyrinogen accumulation and response to porphyrogenic drugs. Can J Physiol Pharmacol. 1997 Oct-Nov;75(10-11):1181-7. [PubMed:9431441 ]
  4. Bloomer JR: Enzyme defects in the porphyrias and their relevance to the biochemical abnormalities in these disorders. J Invest Dermatol. 1981 Jul;77(1):102-6. [PubMed:7252240 ]
  5. de Villiers JN, Kotze MJ, van Heerden CJ, Sadie A, Gardner HF, Liebenberg J, van Zyl R, du Plessis L, Kimberg M, Frank J, Warnich L: Overrepresentation of the founder PPOX gene mutation R59W in a South African patient with severe clinical manifestation of porphyria. Exp Dermatol. 2005 Jan;14(1):50-5. [PubMed:15660919 ]
  6. Corrigall AV, Hift RJ, Adams PA, Kirsch RE: Inhibition of mammalian protoporphyrinogen oxidase by acifluorfen. Biochem Mol Biol Int. 1994 Dec;34(6):1283-9. [PubMed:7697001 ]
  7. Cox TM, Jack N, Lofthouse S, Watling J, Haines J, Warren MJ: King George III and porphyria: an elemental hypothesis and investigation. Lancet. 2005 Jul 23-29;366(9482):332-5. [PubMed:16039338 ]
  8. Winkelman JW, Collins GH: Neurotoxicity of tetraphenylporphinesulfonate TPPS4 and its relation to photodynamic therapy. Photochem Photobiol. 1987 Nov;46(5):801-7. [PubMed:3441503 ]


General function:
Involved in coproporphyrinogen oxidase activity
Specific function:
Key enzyme in heme biosynthesis. Catalyzes the oxidative decarboxylation of propionic acid side chains of rings A and B of coproporphyrinogen III.
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Uniprot ID:
Molecular weight:
Coproporphyrinogen III + Oxygen + Hydrogen Ion → Protoporphyrinogen IX + CO(2) + Waterdetails
Coproporphyrinogen III + Oxygen → Protoporphyrinogen IX + Carbon dioxide + Waterdetails
General function:
Involved in catalytic activity
Specific function:
Catalyzes the 6-electron oxidation of protoporphyrinogen-IX to form protoporphyrin-IX.
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Uniprot ID:
Molecular weight:
Protoporphyrinogen IX + Oxygen → Protoporphyrin IX + Hydrogen peroxidedetails
Protoporphyrinogen IX + Oxygen → Protoporphyrin IX + Hydrogen peroxidedetails