Heme Biosynthesis (Bos taurus)

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1delta-aminolevulinateRed - side productsBlack - reactantsrate limiting stepprotoporphyrinogen IXdelta-aminolevulinateCO2protoporphyrin IXCO2uroporphyrinogen IIIO2H2OO2URODPPOXHMBSH2O2protohemeH2OporphobilinogenNH3CPOXH2OH+CoAsuccinyl-CoAALAS1Fe2+Porphobilinogencoproporphyrinogen IIICO2ALAS2ALADUROShydroxymethylbilaneFECHGlycine


The enzymatic process that produces heme is properly called porphyrin synthesis, as all the intermediates are tetrapyrroles that are chemically classified are porphyrins. The process is highly conserved across biology. In humans, this pathway serves almost exclusively to form heme. In other species, it also produces similar substances such as cobalamin (vitamin B12).

The pathway is initiated by the synthesis of D-Aminolevulinic acid (dALA or δALA) from the amino acid glycine and succinyl-CoA from the citric acid cycle (Krebs cycle). The rate-limiting enzyme responsible for this reaction, ALA synthase, is strictly regulated by intracellular iron levels and heme concentration. A low-iron level, e.g., in iron deficiency, leads to decreased porphyrin synthesis, which prevents accumulation of the toxic intermediates. This mechanism is of therapeutic importance: infusion of heme arginate or hematin can abort attacks of porphyria in patients with an inborn error of metabolism of this process, by reducing transcription of ALA synthase.

The organs mainly involved in heme synthesis are the liver and the bone marrow, although every cell requires heme to function properly. Heme is seen as an intermediate molecule in catabolism of haemoglobin in the process of bilirubin metabolism.

Source: Wikipedia http://en.wikipedia.org/wiki/Heme


This pathway was inferred from Homo sapiens pathway WP561(72035) with a 100.0% conversion rate.

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Ontology Terms



  1. Polo CF, Vazquez ES, Caballero F, Gerez E, Battle AM; ''Heme biosynthesis pathway regulation in a model of hepatocarcinogenesis pre-initiation.''; Comp Biochem Physiol B, 1992 PubMed Europe PMC Scholia


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107076view14:16, 17 September 2019MaintBotChEBI identifier normalization
105974view11:50, 16 August 2019MaintBotHMDB identifier normalization
93012view15:00, 24 July 2017DeSlChanged (vague) CAS to ChEBI ID (which looked to be more relevant).
89919view13:32, 6 October 2016MkutmonModified description
80842view15:28, 30 June 2015Mkutmonhomology conversion
79163view05:17, 6 March 2015MaintBotUpdated conversion from Hs
71851view20:12, 18 October 2013MaintBotremoved data source from nodes without identifier
63475view17:20, 10 May 2013MaintBotupdating gpml
40576view19:32, 1 March 2011MaintBotRemoved redundant pathway information and comments
35292view22:54, 11 February 2010Mills42Modified description
33814view00:22, 9 December 2009MaintBotAutomatic update of empty xrefs
33616view10:01, 1 December 2009MaintBotRemoved group label
30536view21:55, 29 July 2009MaintBotNew pathway

External references


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NameTypeDatabase referenceComment
ALADGeneProductENSBTAG00000000251 (Ensembl) HomologyConvert: Homo sapiens to Bos taurus: Original ID = L:210
ALAS1GeneProductENSBTAG00000004118 (Ensembl) HomologyConvert: Homo sapiens to Bos taurus: Original ID = L:211
ALAS2GeneProductENSBTAG00000013178 (Ensembl) HomologyConvert: Homo sapiens to Bos taurus: Original ID = L:212
CO2MetaboliteHMDB0001967 (HMDB)
CPOXGeneProductENSBTAG00000004124 (Ensembl) HomologyConvert: Homo sapiens to Bos taurus: Original ID = L:1371
CoAMetaboliteHMDB0001423 (HMDB)
FECHGeneProductENSBTAG00000006393 (Ensembl) HomologyConvert: Homo sapiens to Bos taurus: Original ID = L:2235
Fe2+Metabolite27284 (PubChem-compound)
GlycineMetaboliteHMDB0000123 (HMDB)
H+Metabolite1038 (PubChem-compound)
H2O2MetaboliteHMDB0003125 (HMDB)
H2OMetaboliteHMDB0002111 (HMDB)
HMBSGeneProductENSBTAG00000005364 (Ensembl) HomologyConvert: Homo sapiens to Bos taurus: Original ID = L:3145
NH3MetaboliteHMDB0000051 (HMDB)
O2MetaboliteHMDB0001377 (HMDB)
PPOXGeneProductENSBTAG00000021894 (Ensembl) HomologyConvert: Homo sapiens to Bos taurus: Original ID = L:5498
PorphobilinogenMetaboliteHMDB0000245 (HMDB)
URODGeneProductENSBTAG00000012644 (Ensembl) HomologyConvert: Homo sapiens to Bos taurus: Original ID = L:7389
UROSGeneProductENSBTAG00000005791 (Ensembl) HomologyConvert: Homo sapiens to Bos taurus: Original ID = L:7390
coproporphyrinogen IIIMetabolite2624-63-7 (CAS)
delta-aminolevulinateMetabolite10447539 (Chemspider)
hydroxymethylbilaneMetaboliteCHEBI:16645 (ChEBI)
porphobilinogenMetabolite487-90-1 (CAS)
protohemeMetabolite14875-96-8 (CAS)
protoporphyrin IXMetabolite553-12-8 (CAS)
succinyl-CoAMetaboliteHMDB0001022 (HMDB)
uroporphyrinogen IIIMetabolite1976-85-8 (CAS)

Annotated Interactions

No annotated interactions
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