Heme Biosynthesis (Mus musculus)

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protoporphyrinogen IXdelta-aminolevulinateRed - side productsrate limiting stepBlack - reactantssuccinyl-CoA + glycineH+delta-aminolevulinateFe2+FechHmbsNH3O2H2OCoproporphyrinogen IIIProtoporphyrin IXUroporphyrinogen IIIPpoxCO2H2OCO2CpoxAladAlas2HydroxymethylbilaneH2O2UrosAlas1UrodCO2H2OPorphobilinogenprotohemePorphobilinogenCoAO2


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



This pathway was inferred from Homo sapiens pathway WP561(r21860) with a 60% conversion rate.

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107200view14:29, 17 September 2019MaintBotChEBI identifier normalization
106861view13:34, 17 September 2019MaintBotHMDB identifier normalization
93013view15:02, 24 July 2017DeSlChanged (vague) CAS to ChEBI ID (which looked to be more relevant).
89923view13:34, 6 October 2016MkutmonModified description
69996view22:32, 11 July 2013MaintBotupdated to 2013 schema
67334view10:38, 26 June 2013Christine ChichesterOntology Term : 'heme biosynthetic pathway' added !
41372view00:28, 2 March 2011MaintBotRemoved redundant pathway information and comments
35285view22:48, 11 February 2010Mills42Modified description
34418view23:14, 9 December 2009MaintBotAutomatic update of empty xrefs
32667view16:05, 27 August 2009KhanspersAdded CAS metabolites
31989view13:13, 14 August 2009MaintBotFixed group labels
30811view23:55, 29 July 2009MaintBotConverted from Homo sapiens
20713view11:29, 14 November 2008MaintBot[[Pathway:Mus musculus:Heme Biosynthesis]] moved to [[Pathway:WP18]]: Moved to stable identifier
8134view13:46, 7 January 2008MaintBot[[Pathway:Mouse:Heme Biosynthesis]] moved to [[Pathway:Mus musculus:Heme Biosynthesis]]: Renaming species
7688view16:09, 18 December 2007MaintBotfixed category names
7373view12:42, 4 November 2007MaintBotAdded categories to GPML
19493view22:19, 22 May 2007M.Braymergpml file for [[Mouse:Heme_Biosynthesis]]
6456view22:19, 22 May 2007Kdahlquistgpml file for [[Mouse:Heme_Biosynthesis]]

External references


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NameTypeDatabase referenceComment
AladGeneProduct17025 (Entrez Gene)
Alas1GeneProduct11655 (Entrez Gene)
Alas2GeneProduct11656 (Entrez Gene)
CO2MetaboliteHMDB01967 (HMDB)
CoAMetaboliteHMDB01423 (HMDB)
Coproporphyrinogen IIIMetabolite2624-63-7 (CAS)
CpoxGeneProduct12892 (Entrez Gene)
FechGeneProduct14151 (Entrez Gene)
H2O2MetaboliteHMDB03125 (HMDB)
H2OMetaboliteHMDB02111 (HMDB)
HmbsGeneProduct15288 (Entrez Gene)
HydroxymethylbilaneMetabolite73023-76-4 (CAS)
NH3MetaboliteHMDB00051 (HMDB)
O2MetaboliteHMDB01377 (HMDB)
PorphobilinogenMetabolite487-90-1 (CAS)
PpoxGeneProduct19044 (Entrez Gene)
Protoporphyrin IXMetabolite553-12-8 (CAS)
UrodGeneProduct22275 (Entrez Gene)
Uroporphyrinogen IIIMetabolite1976-85-8 (CAS)
UrosGeneProduct22276 (Entrez Gene)
protohemeMetabolite14875-96-8 (CAS)

Annotated Interactions

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