Summary: Porphyrins with four acetic acid and four propionic acid side chains attached to the pyrrole rings.

Top Publications

  1. Sinclair P, Gorman N, Dalton T, Walton H, Bement W, Sinclair J, et al. Uroporphyria produced in mice by iron and 5-aminolaevulinic acid does not occur in Cyp1a2(-/-) null mutant mice. Biochem J. 1998;330 ( Pt 1):149-53 pubmed
    ..These results indicate the absolute requirement for hepatic CYP1A2 in causing experimental uroporphyria under the conditions used. ..
  2. Warren M, Roessner C, Ozaki S, Stolowich N, Santander P, Scott A. Enzymatic synthesis and structure of precorrin-3, a trimethyldipyrrocorphin intermediate in vitamin B12 biosynthesis. Biochemistry. 1992;31:603-9 pubmed
    ..The implications of this structure for corrin biosynthesis are discussed. ..
  3. Deery E, Schroeder S, Lawrence A, Taylor S, Seyedarabi A, Waterman J, et al. An enzyme-trap approach allows isolation of intermediates in cobalamin biosynthesis. Nat Chem Biol. 2012;8:933-40 pubmed publisher
    ..The tight association of pathway intermediates with enzymes provides evidence for a form of metabolite channeling...
  4. Warren M, Raux E, Schubert H, Escalante Semerena J. The biosynthesis of adenosylcobalamin (vitamin B12). Nat Prod Rep. 2002;19:390-412 pubmed
    ..This review covers some of the most important findings that have been made and provides the reader with a complete description of the transformation of uroporphyrinogen III into adenosylcobalamin (AdoCbl). 183 references are cited. ..
  5. Shipman L, Li D, Roessner C, Scott A, Sacchettini J. Crystal structure of precorrin-8x methyl mutase. Structure. 2001;9:587-96 pubmed
    ..Analysis of the structure with bound product suggests that the [1,5]-sigmatropic shift proceeds by protonation of the ring C nitrogen, leading to subsequent methyl migration...
  6. Kajiwara Y, Santander P, Roessner C, P rez L, Scott A. Genetically engineered synthesis and structural characterization of cobalt-precorrin 5A and -5B, two new intermediates on the anaerobic pathway to vitamin B12: definition of the roles of the CbiF and CbiG enzymes. J Am Chem Soc. 2006;128:9971-8 pubmed publisher
  7. Krijt J, Psenak O, Vokurka M, Chlumska A, Fakan F. Experimental hepatic uroporphyria induced by the diphenyl-ether herbicide fomesafen in male DBA/2 mice. Toxicol Appl Pharmacol. 2003;189:28-38 pubmed
  8. Guennoun N, Gerolami Colombani V, Sebti A, Aquaron R. [Sporadic porphyria cutanea tarda: a case report in a Moroccan man]. Med Trop (Mars). 2003;63:183-7 pubmed
    ..The photoprotective effect of melanin in this dark-skinned patient failed to offset uroporphyrin-induced photosensitivity. Avoidance of sun, ethanol and phlebotomy have prevented recurrences. ..
  9. Greaves P, Clothier B, Davies R, Higginson F, Edwards R, Dalton T, et al. Uroporphyria and hepatic carcinogenesis induced by polychlorinated biphenyls-iron interaction: absence in the Cyp1a2(-/-) knockout mouse. Biochem Biophys Res Commun. 2005;331:147-52 pubmed
    ..Our findings suggest a link not only between CYP1A2, iron metabolism, and the induction of uroporphyria by PCBs, but also with subsequent hepatocarcinogenesis. ..

More Information


  1. Krishnamohan M, Qi L, Lam P, Moore M, Ng J. Urinary arsenic and porphyrin profile in C57BL/6J mice chronically exposed to monomethylarsonous acid (MMAIII) for two years. Toxicol Appl Pharmacol. 2007;224:89-97 pubmed
    ..Our results show urinary DMA(V) and porphyrin profile can be used as an early warning biomarker for chronic MMA(III) exposure before the onset of cancer. ..
  2. Lançoni G, Ravinal R, Costa R, Roselino A. Mast cells and transforming growth factor-beta expression: a possible relationship in the development of porphyria cutanea tarda skin lesions. Int J Dermatol. 2008;47:575-81 pubmed publisher
    ..The results suggest that the increased number of mast cells and of cells expressing TGF-beta, as well as their direct correlation, may contribute to the pathogenesis of the skin lesions in PCT. ..
  3. Sattler I, Roessner C, Stolowich N, Hardin S, Harris Haller L, Yokubaitis N, et al. Cloning, sequencing, and expression of the uroporphyrinogen III methyltransferase cobA gene of Propionibacterium freudenreichii (shermanii). J Bacteriol. 1995;177:1564-9 pubmed
    ..These two genes do not appear to constitute part of an extensive cobalamin operon. ..
  4. Gross U, Honcamp M, Daume E, Frank M, Düsterberg B, Doss M. Hormonal oral contraceptives, urinary porphyrin excretion and porphyrias. Horm Metab Res. 1995;27:379-83 pubmed
    ..Treatment with the OCs successfully stabilized the latent phase of premenstrual forms of acute hepatic porphyrias. This study demonstrates that low-dose OCs do not affect urinary porphyrin excretion. ..
  5. Johansson P, Hederstedt L. Organization of genes for tetrapyrrole biosynthesis in gram--positive bacteria. Microbiology. 1999;145 ( Pt 3):529-38 pubmed
    ..It is shown that YInF is required for sirohaem synthesis and probably catalyses the precorrin-2 to sirohaem conversion. YInD probably catalyses precorrin-2 synthesis from UroIII and NasF seems to be specific for nitrite reduction. ..
  6. Negroni R, Arechavala A, Maiolo E. [Clinical cases in medical mycology. Case no. 20]. Rev Iberoam Micol. 2006;23:116-8 pubmed
  7. Saha K, Webb M, Rigby S, Leech H, Warren M, Smith A. Characterization of the evolutionarily conserved iron-sulfur cluster of sirohydrochlorin ferrochelatase from Arabidopsis thaliana. Biochem J. 2012;444:227-37 pubmed publisher
    ..These observations suggest an important physiological role for the Fe-S cluster in Planta, highlighting the close association of iron, sulfur and tetrapyrrole metabolism. ..
  8. De Matteis F. Role of iron in the hydrogen peroxide-dependent oxidation of hexahydroporphyrins (porphyrinogens): a possible mechanism for the exacerbation by iron of hepatic uroporphyria. Mol Pharmacol. 1988;33:463-9 pubmed
    ..These results suggest a possible mechanism for the exacerbation of uroporphyria by excess iron and also for its marked improvement when the iron stores are diminished, for example, by DES treatment. ..
  9. Clavero S, Bishop D, Haskins M, Giger U, Kauppinen R, Desnick R. Feline acute intermittent porphyria: a phenocopy masquerading as an erythropoietic porphyria due to dominant and recessive hydroxymethylbilane synthase mutations. Hum Mol Genet. 2010;19:584-96 pubmed publisher
    ..These animal models may permit further investigation of the pathogenesis of the acute, life-threatening neurological attacks in human AIP and the evaluation of therapeutic strategies. GenBank Accession Numbers: GQ850461-GQ850464. ..
  10. Gorman N, Trask H, Bement W, Szakacs J, Elder G, Balestra D, et al. Genetic factors influence ethanol-induced uroporphyria in Hfe(-/-) mice. Hepatology. 2004;40:942-50 pubmed
    ..In conclusion, this study demonstrates a strong effect of genetic background on ethanol-induced uroporphyria, which is probably due to a greater effect of ethanol on iron metabolism in the susceptible strain. ..
  11. Moore S, Sowa S, Schuchardt C, Deery E, Lawrence A, Ramos J, et al. Elucidation of the biosynthesis of the methane catalyst coenzyme F430. Nature. 2017;543:78-82 pubmed publisher
    ..The research completes our understanding of how the repertoire of tetrapyrrole-based pigments are constructed, permitting the development of recombinant systems to use these metalloprosthetic groups more widely. ..
  12. Schubert H, Rose R, Leech H, Brindley A, Hill C, Rigby S, et al. Structure and function of SirC from Bacillus megaterium: a metal-binding precorrin-2 dehydrogenase. Biochem J. 2008;415:257-63 pubmed publisher
  13. Meng J, Gu H, Zheng R, Xing D, Wang J, Wang R, et al. [The non-trauma fluorescence analysis of the porphyrin in the blood of human superficial skin tissue]. Guang Pu Xue Yu Guang Pu Fen Xi. 2003;23:325-7 pubmed
    ..It was shown that the fluorescence spectrum of the porphyrin obtained from the superficial skin tissue of human earlobe has the same feature as that from the aqueous solution with PP IX. ..
  14. Juzeniene A, Thu Tam T, Iani V, Moan J. 5-Methyltetrahydrofolate can be photodegraded by endogenous photosensitizers. Free Radic Biol Med. 2009;47:1199-204 pubmed publisher
    ..This study suggests that 5MTHF in blood can be photodegraded in the presence of the flavins and porphyrins, but protected by bilirubins. This may have health and evolutionary implications. ..
  15. Palma Carlos A, Palma Carlos M. Solar urticaria and porphyrias. Eur Ann Allergy Clin Immunol. 2005;37:17-20 pubmed
    ..Due to the risk of severe forms of disease sometimes drugs induced porphyrias must be considered in all cases of solar urticaria and a correct laboratory study done in the suspected cases. ..
  16. Wimmershoff F, Gardlo K, Bolsen K, Ruzicka T, Fritsch C. [High-dose vitamin therapy as prophylaxis against porphyria cutanea uremica]. Hautarzt. 2006;57:228, 230-2, 234-6 pubmed
    ..87, as opposed to the PCU group with 3.7. Considerable differences between the level of vitamin ingestion were identified between the groups. Patients with PCU took distinctly less vitamins C, E and B than patients without symptoms. ..
  17. Buchenau B, Kahnt J, Heinemann I, Jahn D, Thauer R. Heme biosynthesis in Methanosarcina barkeri via a pathway involving two methylation reactions. J Bacteriol. 2006;188:8666-8 pubmed
    ..The existence of this pathway, previously exclusively found in the sulfate-reducing delta-proteobacterium Desulfovibrio vulgaris, was demonstrated for M. barkeri via the incorporation of two methyl groups from methionine into protoheme...
  18. Beukeveld G, Wolthers B, Nordmann Y, Deybach J, Grandchamp B, Wadman S. A retrospective study of a patient with homozygous form of acute intermittent porphyria. J Inherit Metab Dis. 1990;13:673-83 pubmed
    ..Due to the early onset in the index patient, its persistent character, and the fact that both parents are affected we postulate retrospectively to have diagnosed a case of homozygous or a double heterozygous AIP, hitherto undescribed. ..
  19. Gu G, Lim C. Preparation and separation of hydroxy derivatives of uroporphyrinogen I by high-performance liquid chromatography with electrochemical detection. J Chromatogr A. 1996;722:245-8 pubmed
    ..The peaks were identified by conversion into the porphyrin methyl esters and analysed by liquid secondary-ion mass spectrometry. ..
  20. Franklin M, Phillips J, Kushner J. CYP3A-inducing agents and the attenuation of uroporphyrin accumulation and excretion in a rat model of porphyria cutanea tarda. Biochem Pharmacol. 2000;60:1325-31 pubmed
  21. el Sayed F, Viraben R, Bazex J, Gorguet B. [Porphyria cutanea tarda and HIV-1 infection. 2 new cases]. Ann Dermatol Venereol. 1993;120:455-7 pubmed
    ..In both patients HIV seropositivity was discovered before PCT was diagnosed. Up to now, none of the hypotheses put forward concerning the exact role played by HIV in the genesis of PCT has been confirmed. ..
  22. Dutta S, Kolli B, Tang A, Sassa S, Chang K. Transgenic Leishmania model for delta-aminolevulinate-inducible monospecific uroporphyria: cytolytic phototoxicity initiated by singlet oxygen-mediated inactivation of proteins and its ablation by endosomal mobilization of cytosolic uroporphyrin. Eukaryot Cell. 2008;7:1146-57 pubmed publisher
  23. Gorman N, Trask H, Robinson S, Sinclair J, Gerhard G, Smith A, et al. Hexachlorobenzene stimulates uroporphyria in low affinity AHR mice without increasing CYP1A2. Toxicol Appl Pharmacol. 2007;221:235-42 pubmed
    ..In conclusion, some minimal basal expression of CYP1A2 is essential for HCB-mediated enhancement of uroporphyria, but increases in CYP1A2 above that level are not essential. ..
  24. Danton M, Lim C. High-performance liquid chromatography/electrospray ionization tandem mass spectrometry of hydroxylated uroporphyrin and urochlorin derivatives formed by photochemical oxidation of uroporphyrinogen I. Biomed Chromatogr. 2007;21:534-45 pubmed
  25. Macours P, Cotton F. Improvement in HPLC separation of porphyrin isomers and application to biochemical diagnosis of porphyrias. Clin Chem Lab Med. 2006;44:1433-40 pubmed
    ..The present RP-HPLC method is specific and efficient for routine analysis of porphyrins in human urine, feces and plasma. ..
  26. Martinelli A, Villanova M, Roselino A, Figueiredo J, Passos A, Covas D, et al. Abnormal uroporphyrin levels in chronic hepatitis C virus infection. J Clin Gastroenterol. 1999;29:327-31 pubmed
    ..The implications of these findings deserve additional investigation. ..
  27. Carrasco Letelier L, Eguren G, de Mello F, Groves P. Preliminary field study of hepatic porphyrin profiles of Astyanax fasciatus (Teleostei, Characiformes) to define anthropogenic pollution. Chemosphere. 2006;62:1245-52 pubmed
    ..fasciatus is a good bio-indicator of exposure to environmental contaminants, and we propose that this abundant fish species be considered as a sentinel organism for monitoring potential disturbances to freshwater ecosystems. ..
  28. Badiu C, Cristofor D, Voicu D, Coculescu M. Diagnostic traps in porphyria: case report and literature review. Rev Med Chir Soc Med Nat Iasi. 2004;108:584-91 pubmed
    ..The clinician should be aware of the protean manifestations of porphyrias and include them in clinical judgment in various situations. ..
  29. Sinclair P, Gorman N, Walton H, Bement W, Szakacs J, Gonzalez F, et al. Relative roles of CYP2E1 and CYP1A2 in mouse uroporphyria caused by acetone. Arch Biochem Biophys. 2000;384:383-90 pubmed
    ..Acetone was not an inducer of hepatic CYP1A2 in the wild-type mice. Although acetone is an inducer of CYP2E1, CYP1A2 appears to have the essential role in acetone-enhancement of uroporphyria. ..
  30. Ding Y, Lin B, Huie C. Binding studies of porphyrins to human serum albumin using affinity capillary electrophoresis. Electrophoresis. 2001;22:2210-6 pubmed
  31. Rom o C, Ladakis D, Lobo S, Carrondo M, Brindley A, Deery E, et al. Evolution in a family of chelatases facilitated by the introduction of active site asymmetry and protein oligomerization. Proc Natl Acad Sci U S A. 2011;108:97-102 pubmed publisher
    ..Whereas retaining colbaltochelatase activity, this protein has acquired a central cavity with the potential to chaperone or transport metals across the periplasmic space, thereby evolving a new use for an ancient protein subunit...
  32. Bonkovsky H. Mechanism of iron potentiation of hepatic uroporphyria: studies in cultured chick embryo liver cells. Hepatology. 1989;10:354-64 pubmed
    ..Induction of 5-aminolevulinate synthase amplifies the porphyrin overproduction. ..
  33. Lobo S, Brindley A, Warren M, Saraiva L. Functional characterization of the early steps of tetrapyrrole biosynthesis and modification in Desulfovibrio vulgaris Hildenborough. Biochem J. 2009;420:317-25 pubmed publisher
    ..This explains why the uroporphyrinogen III synthase is found fused with the methyltransferase, bypassing the need for uroporphyrinogen III decarboxylase activity...
  34. Tam T, Juzeniene A, Steindal A, Iani V, Moan J. Photodegradation of 5-methyltetrahydrofolate in the presence of Uroporphyrin. J Photochem Photobiol B. 2009;94:201-4 pubmed publisher
    ..Our results show that singlet oxygen is likely to be the main intermediate in the photodegradation of 5MTHF mediated by Uro. Our findings may be useful for further studies the evolution of human skin colours. ..
  35. Smith A, Clothier B, Carthew P, Childs N, Sinclair P, Nebert D, et al. Protection of the Cyp1a2(-/-) null mouse against uroporphyria and hepatic injury following exposure to 2,3,7,8-tetrachlorodibenzo-p-dioxin. Toxicol Appl Pharmacol. 2001;173:89-98 pubmed
    ..This study has implications for both the toxicity assessment of TCDD and the hepatic injury seen in PCT patients. ..
  36. Nakano K, Ishizuka M, Sakamoto K, Fujita S. Absolute requirement for iron in the development of chemically induced uroporphyria in mice treated with 3-methylcholanthrene and 5-aminolevulinate. Biometals. 2009;22:345-51 pubmed publisher
    ..We showed that a sufficient level of iron is essential for the development of porphyria and UROX activity. ..
  37. Afonso S, Enriquez De Salamanca R, Batlle A. Photodynamic and light independent action of 8 to 2 carboxylic free porphyrins on some haem-enzymes. Int J Biochem Cell Biol. 2001;33:1208-14 pubmed
    ..On the basis of these results, no correlation between enzyme alterations and the physico-chemical properties of porphyrins could be established. ..
  38. Ficheux H, Levillain P, Lemonnier A. [Direct spectrophotometric determination of urinary uroporphyrin and coproporphyrin using zerocrossing second derivative spectrophotometry]. Ann Biol Clin (Paris). 1989;47:196-201 pubmed
    ..So it may be a very useful technic for clinical biochemistry laboratories in charge of monitoring patients suffering from porphyria. ..
  39. Zajicek R, Bali S, Arnold S, Brindley A, Warren M, Ferguson S. d(1) haem biogenesis - assessing the roles of three nir gene products. FEBS J. 2009;276:6399-411 pubmed publisher
    ..As predicted, NirC is a c-type cytochrome; it was shown in vitro to be an electron donor to the NirN-d(1) complex...
  40. Storbeck S, Walther J, Müller J, Parmar V, Schiebel H, Kemken D, et al. The Pseudomonas aeruginosa nirE gene encodes the S-adenosyl-L-methionine-dependent uroporphyrinogen III methyltransferase required for heme d(1) biosynthesis. FEBS J. 2009;276:5973-82 pubmed publisher
    ..However, bacterial growth of the nirE mutant was observed when cobA was constitutively expressed by a complementing plasmid, underscoring the special requirement of NirE for heme d(1) biosynthesis. ..
  41. el Sharabasy M, el Waseef A, Hafez M, Salim S. Porphyrin metabolism in some malignant diseases. Br J Cancer. 1992;65:409-12 pubmed
    ..The aforementioned disturbances were discussed in the light of some factors which may affect the enzymatic activities in the synthesis of porphyrins. ..
  42. van Birgelen A, Devito M, Akins J, Ross D, Diliberto J, Birnbaum L. Relative potencies of polychlorinated dibenzo-p-dioxins, dibenzofurans, and biphenyls derived from hepatic porphyrin accumulation in mice. Toxicol Appl Pharmacol. 1996;138:98-109 pubmed
    ..However, the higher potencies of the mono-ortho-substituted PCBs have important implications for risk assessment of these compounds. ..
  43. Komagoe K, Tamagake K, Katsu T. The influence of aggregation of porphyrins on the efficiency of photogeneration of hydrogen peroxide in aqueous solution. Chem Pharm Bull (Tokyo). 2006;54:1004-9 pubmed
    ..The present results demonstrated the importance of the state of aggregation of porphyrin to photogenerate H2O2, and the results may help to develop a new type of medicine for photodynamic therapy. ..
  44. Roessner C, Santander P, Scott A. Multiple biosynthetic pathways for vitamin B12: variations on a central theme. Vitam Horm. 2001;61:267-97 pubmed
    ..The aerobic process utilizes two enzymes and is dependent on molecular oxygen, in stark contrast to the anaerobic mechanism which is controlled by cobalt and requires only one enzyme. ..
  45. Park A, Webster G, Penne R, Raber I. Porphyria cutanea tarda presenting as cicatricial conjunctivitis. Am J Ophthalmol. 2002;134:619-21 pubmed
    ..To report a case of porphyria cutanea tarda presenting as cicatricial conjunctivitis...
  46. Sinclair P, Walton H, Gorman N, Jacobs J, Sinclair J. Multiple roles of polyhalogenated biphenyls in causing increases in cytochrome P450 and uroporphyrin accumulation in cultured hepatocytes. Toxicol Appl Pharmacol. 1997;147:171-9 pubmed
    ..We conclude that URO accumulation in chick hepatocyte cultures is limited not only by the induction of CYP1A, but also by the stimulation of intracellular UROGEN oxidation. ..
  47. Akhtar R, Smith A. Chromosomal linkage analysis of porphyria in mice induced by hexachlorobenzene-iron synergism: a model of sporadic porphyria cutanea tarda. Pharmacogenetics. 1998;8:485-94 pubmed
  48. Feliciano J, Liu Y, Daunert S. Novel reporter gene in a fluorescent-based whole cell sensing system. Biotechnol Bioeng. 2006;93:989-97 pubmed
    ..Advantages and limitations of using the cobA gene in whole-cell sensing applications are presented. ..
  49. Bozek P, Hutta M, Hrivnáková B. Rapid analysis of porphyrins at low ng/l and microg/l levels in human urine by a gradient liquid chromatography method using octadecylsilica monolithic columns. J Chromatogr A. 2005;1084:24-32 pubmed
    ..Recovery of porphyrins at low microg/l concentration levels was from 93% to 97.5%. Devised method increases productivity of clinical laboratory from 2 to 10 times in dependence of duration of currently used method. ..
  50. Dutta S, Waki K, Chang K. Combinational sensitization of Leishmania with uroporphyrin and aluminum phthalocyanine synergistically enhances their photodynamic inactivation in vitro and in vivo. Photochem Photobiol. 2012;88:620-5 pubmed publisher
    ..The results suggest that double photosensitization for synergistic activity enhances the efficacy and safety of photodynamic therapy in general and for Leishmania in particular. ..
  51. Adjarov D, Kerimova M. Effective control of patients with porphyria cutanea tarda by measuring plasma uroporphyrin. Clin Exp Dermatol. 1991;16:254-7 pubmed
    ..Retreatment is necessary when the levels exceed 23 nmol/l. The use of the method is recommended as a simple and effective way for monitoring the progress of patients with PCT. ..
  52. Nichols R, Cooper S, Trask H, Gorman N, Dalton T, Nebert D, et al. Uroporphyrin accumulation in hepatoma cells expressing human or mouse CYP1A2: relation to the role of CYP1A2 in human porphyria cutanea tarda. Biochem Pharmacol. 2003;65:545-50 pubmed
    ..Cells expressing mouse CYP1A2 produced more URO than cells expressing human CYP1A2. The results indicate that human CYP1A2 can support URO accumulation in hepatoma cells and thus may play a role in human PCT. ..
  53. Lim C, Danton M, Clothier B, Smith A. Dihydroxy-, hydroxyspirolactone-, and dihydroxyspirolactone-urochlorins induced by 2,3,7,8-tetrachlorodibenzo-p-dioxin in the liver of mice. Chem Res Toxicol. 2006;19:1660-7 pubmed
    ..As well as the major constituents uroporphyrins I and III, we identified the isomers of heptacarboxylic, hexacarboxylic, and pentacarboxylic acid porphyrins ..