Heme biosynthesis

Peoc'h K, Martin-Schmitt C, Talbi N, Deybach JC, Gouya L, Puy H. [Porphyrias and haem related disorders]. Rev Med Interne. 2016 Mar;37(3):173–85. doi: 10.1016/j.revmed.2015.12.005. PMID: 26774916.; Layer G, Reichelt J, Jahn D, Heinz DW. Structure and function of enzymes in heme biosynthesis. Protein Sci. 2010 Jun;19(6):1137–61. PMID: 20506125; PMCID: PMC2895239.; Ajioka RS, Phillips JD, Kushner JP. Biosynthesis of heme in mammals. Biochimica et Biophysica Acta (BBA) - Molecular Cell Research. 2006 Jul;1763(7):723–36. doi: 10.1016/j.bbamcr.2006.05.005.; Ponka P. Cell Biology of Heme. The American Journal of the Medical Sciences. 1999 Oct;318(4):241–56. doi: 10.1016/s0002-9629(15)40628-7.

Metabolites

CO2

Formula: CO2 (43.98983)

CAS ID: 124-38-9

Fe2+

Formula: Fe (55.934939)

CAS ID: 15438-31-0

H+

Formula: H (1.0078246)

CAS ID: 12408-02-5

H2O

Formula: H2O (18.0105642)

CAS ID: 7732-18-5

Hydrogen peroxide

Formula: H2O2 (34.0054792)

CAS ID: 7722-84-1

Lead

Formula: Pb (207.976641)

CAS ID: 7439-92-1

Oxygen

Formula: O2 (31.98983)

CAS ID: 7782-44-7

Zinc cation

Formula: Zn (63.929145)

CAS ID: 23713-49-7

dipyrromethane

Formula: C9H10N2 (146.084394)

CAS ID: 21211-65-4



Enzyme

EC Number name full name note
1.17.99.9 heme a synthase ferroheme o:acceptor C-81-oxidoreductase (heme a-forming)
1.3.3.3 coproporphyrinogen oxidase coproporphyrinogen:oxygen oxidoreductase (decarboxylating)
1.3.3.4 protoporphyrinogen oxidase protoporphyrinogen-IX:oxygen oxidoreductase
2.3.1.37 5-aminolevulinate synthase succinyl-CoA:glycine C-succinyltransferase (decarboxylating)
2.5.1.141 heme o synthase (2E,6E)-farnesyl-diphosphate:protoheme IX farnesyltranstransferase
2.5.1.61 hydroxymethylbilane synthase porphobilinogen:(4-[2-carboxyethyl]-3-[carboxymethyl]pyrrol-2-yl)methyltransferase (hydrolysing)
4.1.1.37 uroporphyrinogen decarboxylase uroporphyrinogen-III carboxy-lyase (coproporphyrinogen-III-forming)
4.2.1.24 porphobilinogen synthase 5-aminolevulinate hydro-lyase (adding 5-aminolevulinate and cyclizing; porphobilinogen-forming)
4.2.1.75 uroporphyrinogen-III synthase hydroxymethylbilane hydro-lyase (cyclizing; uroporphyrinogen-III-forming)
4.98.1.1 protoporphyrin ferrochelatase protoheme ferro-lyase (protoporphyrin-forming)


Proteins

Protein ID name full name
O14092 hem1 5-aminolevulinate synthase, mitochondrial
O59786 hem15 Ferrochelatase, mitochondrial
P78974 hem2 Delta-aminolevulinic acid dehydratase
P87214 ups1 Uroporphyrinogen-III synthase
Q09899 hem3 Porphobilinogen deaminase
Q10062 hem14 Protoporphyrinogen oxidase
Q10361 etp1 Heme A synthase-mitochondrial ferredoxin fusion protein
Q9USJ5 hem12 Uroporphyrinogen decarboxylase
Q9UTE2 hem13 Probable oxygen-dependent coproporphyrinogen-III oxidase
Q9Y7Y4 cox10 Protoheme IX farnesyltransferase, mitochondrial