Heme Biosynthesis (Anopheles gambiae)

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delta-aminolevulinateRed - side productsrate limiting stepH+protoporphyrinogen IXBlack - reactantsdelta-aminolevulinateFe2+succinyl-CoAH2Ocoproporphyrinogen IIIAgaP_AGAP011895CO2CoAH2O2O2glycineprotohemeAgaP_AGAP010935CO2AgaP_AGAP004749AgaP_AGAP003704H2Oprotoporphyrin IXALAS2NH3AgaP_AGAP011080O2H2OAgaP_AGAP008064hydroxymethylbilaneAgaP_AGAP003184porphobilinogenAgaP_AGAP003719CO2Porphobilinogenuroporphyrinogen III


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 53% conversion rate.

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105885view07:47, 16 August 2019MkutmonHMDB identifier unification
105749view14:56, 15 August 2019MkutmonReverted to version '14:50, 12 August 2016' by Mkutmon
105748view14:53, 15 August 2019MkutmonHMDB identifier unification
88637view14:50, 12 August 2016JmeliusOntology Term : 'heme biosynthetic pathway' added !
86923view11:43, 15 July 2016Mkutmonupdate description
71942view21:51, 23 October 2013EgonwConverted five labels into Metabolite DataNodes.
71941view21:49, 23 October 2013EgonwRemoved a copy.
71940view21:49, 23 October 2013EgonwSplit up a label with two metabolites into two DataNodes.
71855view20:15, 18 October 2013MaintBotUpdated data sources
70198view04:31, 13 July 2013MaintBotupdated to 2013 schema
40463view18:46, 1 March 2011MaintBotRemoved redundant pathway information and comments
35294view22:55, 11 February 2010Mills42Modified description
33730view23:42, 8 December 2009MaintBotAutomatic update of empty xrefs
31828view12:26, 14 August 2009MaintBotFixed group labels
30790view23:38, 29 July 2009MaintBotNew pathway

External references


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NameTypeDatabase referenceComment
AgaP_AGAP003184GeneProduct1273856 (Entrez Gene)
AgaP_AGAP003704GeneProduct1274381 (Entrez Gene)
AgaP_AGAP003719GeneProduct1271452 (Entrez Gene)
AgaP_AGAP004749GeneProduct1278476 (Entrez Gene)
AgaP_AGAP008064GeneProduct1277887 (Entrez Gene)
AgaP_AGAP010935GeneProduct1271017 (Entrez Gene)
AgaP_AGAP011080GeneProduct3291232 (Entrez Gene)
AgaP_AGAP011895GeneProduct1280765 (Entrez Gene)
CO2MetaboliteHMDB0001967 (HMDB)
CoAMetaboliteHMDB0001423 (HMDB)
H2O2MetaboliteHMDB0003125 (HMDB)
H2OMetaboliteHMDB0002111 (HMDB)
NH3MetaboliteHMDB0000051 (HMDB)
O2MetaboliteHMDB0001377 (HMDB)
PorphobilinogenMetaboliteHMDB0000245 (HMDB)
coproporphyrinogen IIIMetabolite
protoporphyrin IXMetabolite
protoporphyrinogen IXMetabolite
uroporphyrinogen IIIMetabolite

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