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The final step of heme biosynthesis is the insertion of iron into protoporphyrin IX (OMIM 612386). This reaction is catalyzed by the mitochondrial enzyme ferrochelatase (see Fig. 1). Ferrochelatase utilizes protoporphyrin IX, rather than its reduced form, as substrate, but requires the reduced ferrous form of iron .
Nov 3, 2014 · Under conditions of iron deficiency, lead poisoning or excessive formation of PP, zinc protoporphyrin (ZnPP) is formed as a by-product in the heme biosynthesis pathway, resulting from insertion of Zn 2+ rather than Fe 2+ into protoporphyrin by FECH. FECH is also capable of inserting cobalt into PP with the formation of cobalt protoporphyrin ...
- Siddesh Besur, Weihong Hou, Paul Schmeltzer, Herbert L. Bonkovsky
- 10.3390/metabo4040977
- 2014
- Metabolites. 2014 Dec; 4(4): 977-1006.
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How is uroporphyrinogen III converted into heme?
Which enzymatic steps lead to the biosynthesis of protoporphyrin IX (Proto)?
Is heme a ferrous protoporphyrin?
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May 1, 2023 · Go to: Cellular Level. Porphyrin synthesis is the process that produces heme. Heme synthesis occurs partly in the mitochondria and partly in the cytosol. The biosynthesis involves an eight-step enzymatic pathway. Heme biosynthesis starts in mitochondria with the condensation of succinyl Co-A from the citric acid cycle and an amino acid glycine.
- Aminat S. Ogun, Neena V. Joy, Menogh Valentine
- 2023/05/01
- 2021
Fig. 1 Diagram of heme biosynthetic pathway, showing that a defect in any step causes excess accumulation of porphyrin or porphyrin precursors. * = Hydroxymethylbilane can spontaneously convert into uroporphyrin I and subsequently into coproporphyrin I. ALA = ∂-aminolevulinic acid; CoA = coenzyme A; PBG = porphobilinogen. View Large Image.
- Ayalew Tefferi, Lawrence A. Solberg, Ralph D. Ellefson
- 1994
May 24, 2010 · Depending on the organism, ALA is either synthesized by condensation of glycine with succinyl-CoA or from tRNA-bound glutamate via glutamate-1-semialdehyde. B: Uroporphyrinogen III is converted into heme in four consecutive enzymatic steps via the intermediates coproporphyrinogen III, protoporphyrinogen IX, and protoporphyrin IX.
- Gunhild Layer, Joachim Reichelt, Dieter Jahn, Dirk W. Heinz
- 2010
Sep 5, 2018 · The general precursor molecule of all tetrapyrroles 5-aminolevulinic acid is formed by two different pathways starting either with glutamyl-tRNA or succinyl-CoA and glycine. The conversion of 5-aminolevulinic acid to uroporphyrinogen III is common to all biosynthetic paths.
May 3, 2019 · In photosynthetic organisms, several enzymatic steps of TBS lead to the biosynthesis of protoporphyrin IX (Proto), which is a common substrate for ferrochelatase (FeCh) and Mg-chelatase (MgCh),...