Haem Biosynthesis

The haem biosynthesis pathway consists of a series of enzymatic reactions providing the cells with haem, the end product, and intermediates used as precursors for the synthesis of other tetrapyrrolic pigments, such as the chlorophylls.

Keywords: haemoproteins; porphyrins; chlorophylls; iron; erythropoiesis

Figure 1. Structure of haem (protohaem). In the text, haem is used as a generic term, without specifying whether iron is reduced or oxidized.
Figure 2. Haem biosynthetic pathway in animals. The side chain substituents on the porphyrin macrocycle are A, acetyl; M, methyl; P, propionyl; V, vinyl.
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 References
    Al-Karadaghi S, Hansson M, Nikonov S, Jonsson B and Hederstedt L (1997) Crystal structure of ferrochelatase: the terminal enzyme in heme biosynthesis. Structure 5: 1501–1510.
    Erskine PT, Senior N, Awan S et al. (1997) X-ray structure of 5-aminolaevulinate dehydratase, a hybrid aldolase. Nature Structural Biology 4: 1025–1031.
    Harigae H, Suwabe N, Weinstock PH et al. (1998) Deficient heme and globin synthesis in embryonic stem cells lacking the erythroid-specific 5-aminolevulinate synthase gene. Blood 91: 798–805.
    Louie GV, Brownlie PD, Lambert R et al. (1996) The three-dimensional structure of Escherichia coli porphobilinogen deaminase at 1.76-Å resolution. Proteins Structure Function and Genetics 25: 48–78.
    Whitby FG, Phillips JD, Kushner JP and Hill CP (1998) Crystal structure of human uroporphyrinogen decarboxylase. EMBO Journal 17: 2463–2471.
 Further Reading
    book Chadwick DL and Ackrill K (eds) (1994) The Biosynthesis of the Tetrapyrrole Pigments. Ciba Foundation Symposium 180. Chichester: Wiley.
    book Dailey HA (ed.) (1990) Biosynthesis of Heme and Chlorophylls. New York: McGraw-Hill.
    Ferreira GC (Series ed.) (1995) Heme biosynthesis: biochemistry, molecular biology, and relationship to disease. Journal of Bioenergetics and Biomembranes 27: 147–248.
    Hentze M and Kuhn LC (1996) Molecular control of vertebrate iron metabolism: mRNA-based regulatory circuits operated by iron, nitric oxide, and oxidative stress. Proceedings of the National Academy of Sciences of the USA 93: 8175–8182.
    book Jordan PM (ed.) (1991) Biosynthesis of Tetrapyrroles. New York: Elsevier Science.
    May BK, Dogra SC, Sadlon TJ et al. (1995) Molecular regulation of heme biosynthesis in higher vertebrates. Progress in Nucleic Acid Research and Molecular Biology 51: 1–51.
    Ponka P (1997) Tissue-specific regulation of iron metabolism and heme synthesis: distinct control mechanisms in erythroid cells. Blood 89: 1–25.
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Labbe‐Bois, Rosine, and Beaumont, Carole(Apr 2001) Haem Biosynthesis. In: eLS. John Wiley & Sons Ltd, Chichester. http://www.els.net [doi: 10.1038/npg.els.0000556]