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P09601 UniProt 7722-84-1 CAS 439161 PubChem-compound C00430 KEGG Compound 937 ChemSpider Porphobilinogen deaminase CHEBI:16134 ChEBI UROPORPHYRIN_III BioCyc COPROPORPHYRINOGEN_I BioCyc 29936 PubChem-compound 439153 PubChem-compound SMILES CCC1=C(C)C(=O)NC1CC1=C(C)C(CCC(O)=O)=C(CC2=C(CCC(O)=O)C(C)=C(CC3NC(=O)C(C=C)=C3C)N2)N1 HYDROXYMETHYLBILANE BioCyc C00448 KEGG Compound PW000174 PathWhiz Uroporphyrinogen III 607-14-7 CAS 1.0 1146 ChemSpider CHEBI:15380 ChEBI NADH C25H40N7O19P3S Succinyl-CoA 867.1312 CHEBI:17200 ChEBI 952 ChemSpider ALPHA-GLUCOSE-16-BISPHOSPHATE BioCyc HMDB06901 HMDB HMDB00123 HMDB Acute Intermittent Porphyria HMDB00245 HMDB UDP-GLUCURONATE BioCyc 6031 PubChem-compound PW000157 PathWhiz 1.0 CHEBI:16240 ChEBI 1.0 445713 PubChem-compound HMDB00241 HMDB P10746 UniProt 1.0 27815 ChemSpider P22830 UniProt Fe2+ Uridine 5'-diphosphate 531-14-6 CAS CHEBI:27484 ChEBI SMILES CC(C)(COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2N)C(O)C(=O)NCCC(=O)NCCSC(=O)CCC(O)=O C45H52N4O18 Bilirubin diglucuronide 936.32764 389645 ChemSpider C40H38N4O16 Uroporphyrin I 830.2283 5-aminolevulinate synthase, nonspecific, mitochondrial 389644 ChemSpider C15H22N2O18P2 Uridine diphosphate glucuronic acid 580.0343 C00237 KEGG Compound C36H38N4O8 Coproporphyrin I 654.269 389649 ChemSpider C34H40N4O4 Protoporphyrinogen IX 568.30493 Cytosol CHEBI:17381 ChEBI HMDB00570 HMDB 1.0 HMDB00692 HMDB BILIRUBIN BioCyc SMP00344 SMPDB 1.0 C00007 KEGG Compound 1.0 C00001 KEGG Compound C00004 KEGG Compound CHEBI:17033 ChEBI C00003 KEGG Compound C00486 KEGG Compound Protoporphyrin IX P13196 UniProt 114-25-0 CAS Uroporphyrinogen-III synthase UDP-glucuronosyltransferase 2B11 Porphobilinogen deaminase Mitochondrial Inner Membrane Protoporphyrinogen IX 1.0 Protoporphyrinogen oxidase Transport178 false Coproporphyrinogen III (→) Transport: Homo sapiens, Cell, Cytosol to Homo sapiens, Cell, Mitochondrion LEFT_TO_RIGHT C21H28N7O14P2 NAD 664.11694 C00011 KEGG Compound CHEBI:17245 ChEBI C00015 KEGG Compound C00014 KEGG Compound Oxygen SMILES CC(C)=CCC\C(C)=C\CC\C(C)=C\CCC(O)C1=C(C)/C2=C/C3=N/C(=C\C4=C(CCC(O)=O)C(C)=C5/C=C6\N=C(\C=C\1/N\2[Fe]N45)C(C)=C6C=C)/C(CCC(O)=O)=C3C Uroporphyrinogen decarboxylase Ferritin, mitochondrial Coproporphyrinogen-III oxidase, mitochondrial 5-Aminolevulinic acid SMP00004 SMPDB Transport179 false Protoporphyrin IX (→) Transport: Homo sapiens, Cell, Mitochondrial Inner Membrane to Homo sapiens, Cell, Mitochondrial Matrix LEFT_TO_RIGHT 1.0 Q12887 UniProt CHEBI:17499 ChEBI 388307 ChemSpider C40H44N4O16 Uroporphyrinogen III 836.2752 18273-06-8 CAS CPD-10134 BioCyc C40H38N4O16 Uroporphyrin III 830.2283 C36H44N4O8 Coproporphyrinogen III 660.3159 Heme Transport180 false Heme (→) Transport: Homo sapiens, Cell, Mitochondrial Matrix to Homo sapiens, Cell, Cytosol LEFT_TO_RIGHT 53-84-9 CAS O75310 UniProt C00027 KEGG Compound 553-12-8 CAS 1.0 Uroporphyrinogen I 3.0 Fe Fe2+ 55.93494 ReactionCatalysis1717 ACTIVATION CL:0000000 CELL TYPE ONTOLOGY 73023-76-4 CAS ReactionCatalysis1716 ACTIVATION 372-97-4 CAS C00032 KEGG Compound ReactionCatalysis1718 ACTIVATION Uroporphyrin III UDP-glucuronosyltransferase 2B11 C00037 KEGG Compound CHEBI:18276 ChEBI Heme oxygenase 1 SMILES NC(=O)C1=CN(C=CC1)[C@@H]1O[C@H](CO[P@](O)(=O)O[P@](O)(=O)OC[C@H]2O[C@H]([C@H](O)[C@@H]2O)N2C=NC3=C(N)N=CN=C23)[C@@H](O)[C@H]1O Biliverdin reductase A 4.0 Beta-glucuronidase C00282 KEGG Compound 4.0 CHEBI:18392 ChEBI P50336 UniProt HYDROGEN-PEROXIDE BioCyc ReactionCatalysis1726 ACTIVATION AH2 C33H34N4O6 Biliverdin 582.24786 4.0 C02469 KEGG Compound 767 ChemSpider C00167 KEGG Compound 763 ChemSpider Alcohol 604-98-8 CAS 762 ChemSpider 3.0 C9H14N2O12P2 Uridine 5'-diphosphate 404.0022 784 PubChem-compound C15H28O7P2 Farnesyl pyrophosphate 382.131 C40H46N4O17 Hydroxymethylbilane 854.28577 1.0 C15672 KEGG Compound 788 PubChem-compound C15670 KEGG Compound C01024 KEGG Compound Uroporphyrinogen-III synthase Heme A 7732-18-5 CAS BILIRUBIN-BISGLUCURONOSIDE BioCyc Coproporphyrin I 315 ChemSpider ReactionCatalysis1741 ACTIVATION PROTOPORPHYRINOGEN BioCyc 17208-65-0 CAS AMMONIA BioCyc SMILES N C5H9NO3 5-Aminolevulinic acid 131.05824 CHEBI:29033 ChEBI Heme 2.0 CHEBI:29034 ChEBI 16522 ChemSpider Fe Fe3+ 55.93494 Porphobilinogen 5353439 PubChem-compound NAD HMDB00643 HMDB 56-40-6 CAS Bilirubin diglucuronide 4.0 SMILES CC1=C(C=C)\C(NC1=O)=C\C1=C(C)C(CCC(O)=O)=C(CC2=C(CCC(O)=O)C(C)=C(N2)\C=C2/NC(=O)C(C=C)=C2C)N1 FARNESYL-PP BioCyc 630-08-0 CAS SMILES OC(=O)CCC1=C2CC3=C(CC(O)=O)C(CCC(O)=O)=C(CC4=C(CC(O)=O)C(CCC(O)=O)=C(CC5=C(CC(O)=O)C(CCC(O)=O)=C(CC(N2)=C1CC(O)=O)N5)N4)N3 Ferritin, mitochondrial Coproporphyrinogen III SMILES CC1=C(CCC(O)=O)/C2=C/C3=N/C(=C\C4=C(C)C(C=C)=C(N4)/C=C4\N=C(\C=C\1/N\2)C(C=C)=C4C)/C(C)=C3CCC(O)=O A SMILES NCC1=C(CC(O)=O)C(CCC(O)=O)=CN1 HMDB01967 HMDB 1.0 962 PubChem-compound Protoheme IX farnesyltransferase, mitochondrial UROPORPHYRINOGEN-III BioCyc C05767 KEGG Compound Uroporphyrinogen decarboxylase 121893 PubChem-compound 58-98-0 CAS SubPathwayInteraction639 SubPathway639Reaction SubPathwayReaction C05766 KEGG Compound 1.0 C05769 KEGG Compound C34H32FeN4O4 Heme 616.1773 CO2 Carbon dioxide 43.98983 C05768 KEGG Compound Coproporphyrinogen III C05770 KEGG Compound 730 ChemSpider SMILES O=O 2.0 C01051 KEGG Compound SMILES CC(C)=CCC\C(C)=C\CC\C(C)=C\CCC(O)C1=C2C=C3C(C)=C(C=C)C4=[N]3[Fe++]35N6C(=C4)C(C)=C(CCC(O)=O)C6=CC4=[N]3C(=CC(N25)=C1C)C(C=O)=C4CCC(O)=O Hydrogen peroxide BiologicalState3 5809 ChemSpider BiologicalState2 1.0 Ferrochelatase, mitochondrial Ferrochelatase, mitochondrial Delta-aminolevulinic acid dehydratase C00091 KEGG Compound 4.0 C36H44N4O8 Coproporphyrinogen I 660.3159 C36H38N4O8 Coproporphyrin III 654.269 C05787 KEGG Compound Glycine SubPathwayOutput 995 ChemSpider C05791 KEGG Compound ReactionCatalysis1700 ACTIVATION ReactionCatalysis1702 ACTIVATION H2O Water 18.010565 H2O2 Hydrogen peroxide 34.005478 SMILES NCC(O)=O HMDB12943 HMDB Reaction1911 false Heme O → Heme A LEFT_TO_RIGHT 1867-62-5 CAS NADH BioCyc NAD BioCyc 15438-31-0 CAS Farnesyl pyrophosphate 1.0 1.0 HMDB00961 HMDB 1.0 UROPORPHYRIN_I BioCyc 977 PubChem-compound 31110-56-2 CAS 25394 ChemSpider 1.0 ReactionCatalysis1706 ACTIVATION C01079 KEGG Compound 2.0 ReactionCatalysis1709 ACTIVATION 5-AMINO-LEVULINATE BioCyc C03263 KEGG Compound ReactionCatalysis1710 ACTIVATION 1.0 10469486 ChemSpider 1.0 2.5.1.- false 2.5.1.- Farnesyl pyrophosphate + Heme → Heme O LEFT_TO_RIGHT COPROPORPHYRINOGEN_III BioCyc SMILES [H][H] Carbon monoxide 16736727 ChemSpider 16736725 ChemSpider Glycine and Serine Metabolism SubPathway Cytochrome c oxidase assembly protein COX15 homolog SMILES CC1=C(CCC(O)=O)/C2=C/C3=N/C(=C\C4=C(C)C(CCC(O)=O)=C(N4)/C=C4\N=C(\C=C\1/N\2)C(C)=C4CCC(O)=O)/C(CCC(O)=O)=C3C 134 ChemSpider SMILES O SMILES OO 3.2.1.31 false 3.2.1.31 Bilirubin diglucuronide + Water → Alcohol + D-Urobilinogen LEFT_TO_RIGHT H3N Ammonia 17.026548 Coproporphyrinogen I 58838673 PubChem-compound BILIVERDINE BioCyc 2.4.1.17 false 2.4.1.17 Bilirubin + Uridine diphosphate glucuronic acid → Bilirubin diglucuronide + Uridine 5'-diphosphate LEFT_TO_RIGHT C33H36N4O6 Bilirubin 584.2635 2616-64-0 CAS 1.3.1.24 false 1.3.1.24 Biliverdin + NAD → Bilirubin + NADH LEFT_TO_RIGHT Protoporphyrinogen oxidase Beta-glucuronidase ReactionCatalysis1699 ACTIVATION 393270 ChemSpider C40H44N4O16 Uroporphyrinogen I 836.2752 C02191 KEGG Compound 1.0 5280352 PubChem-compound 1.14.99.3 false 1.14.99.3 3 AH2 + Heme + 3 Oxygen → 3 A + Biliverdin + Carbon monoxide + Fe2+ + 3 Water LEFT_TO_RIGHT 3571849 ChemSpider CHEBI:15846 ChEBI C10H14N2O4 Porphobilinogen 226.09535 HMDB00935 HMDB HMDB00936 HMDB 2.0 C34H34N4O4 Protoporphyrin IX 562.258 16736701 ChemSpider SMILES [Fe++] HMDB04158 HMDB GO:0005829 GENE ONTOLOGY 275 ChemSpider 274 ChemSpider HMDB00902 HMDB Ferric-Hydroxamate-Complexes BioCyc 4510089 ChemSpider CHEBI:16908 ChEBI 137 PubChem-compound Ammonia 4.2.1.75 false 4.2.1.75 Hydroxymethylbilane → Uroporphyrinogen III + Water LEFT_TO_RIGHT SMILES CC1=C(C=C)\C(NC1=O)=C\C1=C(C)C(CCC(O)=O)=C(N1)\C=C1/N\C(=C\C2=NC(=O)C(C=C)=C2C)C(C)=C1CCC(O)=O 1021 PubChem-compound 137397-56-9 CAS 7412-77-3 CAS SMILES O[C@H]1[C@@H](O)[C@@H](O[C@@H]1COP(O)(=O)OP(O)(O)=O)N1C=CC(=O)NC1=O P36551 UniProt Coproporphyrin III Mitochondrion HMDB00916 HMDB 1.0 1.0 GLY BioCyc Carbon dioxide 27284 PubChem-compound P08397 UniProt CPD-6641 BioCyc 321 PubChem-compound 217 ChemSpider 58-68-4 CAS 108741 ChemSpider Water ReactionCatalysis1766 ACTIVATION CHEBI:24480 ChEBI ReactionCatalysis1765 ACTIVATION ReactionCatalysis1767 ACTIVATION 124-38-9 CAS C2H5NO2 Glycine 75.03203 Fe2+ 4.0 3.0 1.0 HMDB03178 HMDB 1.0 635-65-4 CAS 1.0 Q8N4E7 UniProt Heme O 5257127 PubChem-compound GO:0005759 GENE ONTOLOGY 17473 PubChem-compound Hydrogen 7664-41-7 CAS 1.0 Hydroxymethylbilane CO Carbon monoxide 27.994915 Succinyl-CoA H2 Hydrogen 2.01565 C00931 KEGG Compound 4444055 ChemSpider Reaction1879 false Hydroxymethylbilane → Uroporphyrinogen I LEFT_TO_RIGHT 1.0 SMILES O=C=O SMILES CC1=C(CCC(O)=O)C2=CC3=[N+]4C(=CC5=C(C=C)C(C)=C6C=C7C(C=C)=C(C)C8=[N+]7[Fe@]4(N2C1=C8)N56)C(C)=C3CCC(O)=O CHEBI:27609 ChEBI 222 PubChem-compound HMDB01086 HMDB C21H29N7O14P2 NADH 665.12476 Reaction1877 false Uroporphyrinogen III → Uroporphyrin III LEFT_TO_RIGHT GO:0005743 GENE ONTOLOGY 1.0 17459-92-6 CAS 2.5.1.61 false 2.5.1.61 4 Porphobilinogen + Water → 4 Ammonia + Hydroxymethylbilane LEFT_TO_RIGHT 2.3.1.37 false 2.3.1.37 Glycine + Succinyl-CoA → 5-Aminolevulinic acid + Carbon dioxide LEFT_TO_RIGHT 16736509 ChemSpider 4.2.1.24 false 4.2.1.24 5-Aminolevulinic acid → Porphobilinogen + 2 Water LEFT_TO_RIGHT ReactionCatalysis1783 ACTIVATION 1.0 1.0 Hydrogen HMDB01097 HMDB SMILES CC1=C2CC3=C(CCC(O)=O)C(C)=C(CC4=C(CCC(O)=O)C(C)=C(CC5=C(CCC(O)=O)C(C)=C(CC(N2)=C1CCC(O)=O)N5)N4)N3 1.0 GO:0005739 GENE ONTOLOGY 3.0 PORPHOBILINOGEN BioCyc Biliverdin HMDB02158 HMDB Heme O HMDB03125 HMDB CHEBI:17627 ChEBI P08236 UniProt 3.0 A SMILES NC(=O)C1=C[N+](=CC=C1)[C@@H]1O[C@H](COP(O)(=O)OP(O)(=O)OC[C@H]2O[C@H]([C@H](O)[C@@H]2O)N2C=NC3=C2N=CN=C3N)[C@@H](O)[C@H]1O 3.0 D-Urobilinogen Protoporphyrin IX 5682 ChemSpider UDP BioCyc 1.3.3.4 false 1.3.3.4 3 Oxygen + Protoporphyrinogen IX → 3 Hydrogen peroxide + Protoporphyrin IX LEFT_TO_RIGHT 440784 PubChem-compound CHEBI:28766 ChEBI 14643-66-4 CAS 14875-96-8 CAS CHEBI:15439 ChEBI 1.16.3.1 false 1.16.3.1 4 Fe2+ + 4 Hydrogen + Oxygen ← 4 Fe3+ + 2 Water RIGHT_TO_LEFT Mitochondrial Matrix 4.99.1.1 false 4.99.1.1 Fe2+ + Protoporphyrin IX → Heme + 2 Hydrogen LEFT_TO_RIGHT P13716 UniProt Delta-aminolevulinic acid dehydratase SMILES OC(=O)CCC1=C2CC3=C(CCC(O)=O)C(CC(O)=O)=C(CC4=C(CC(O)=O)C(CCC(O)=O)=C(CC5=C(CC(O)=O)C(CCC(O)=O)=C(CC(N2)=C1CC(O)=O)N5)N4)N3 AH2 SMILES OC(=O)CCC1=C(CC(O)=O)/C2=C/C3=N/C(=C\C4=C(CCC(O)=O)C(CC(O)=O)=C(N4)/C=C4\N=C(\C=C\1/N\2)C(CC(O)=O)=C4CCC(O)=O)/C(CCC(O)=O)=C3CC(O)=O SMILES CC1=C2CC3=C(C)C(CCC(O)=O)=C(CC4=C(CCC(O)=O)C(C)=C(CC5=C(CCC(O)=O)C(C)=C(CC(N2)=C1CCC(O)=O)N5)N4)N3 440776 PubChem-compound 440775 PubChem-compound Carbon dioxide 1.3.3.3 false 1.3.3.3 Coproporphyrinogen III + 2 Hydrogen + Oxygen → 2 Carbon dioxide + Protoporphyrinogen IX + 2 Water LEFT_TO_RIGHT Fe3+ 2.0 Reaction1886 false Coproporphyrinogen III → Coproporphyrin III LEFT_TO_RIGHT Reaction1884 false Uroporphyrinogen I → Uroporphyrin I LEFT_TO_RIGHT 4.1.1.37 false 4.1.1.37 Uroporphyrinogen III → 4 Carbon dioxide + Coproporphyrinogen III LEFT_TO_RIGHT 57560-10-8 CAS Cell Reaction1883 false Coproporphyrinogen I → Coproporphyrin I LEFT_TO_RIGHT 5459911 PubChem-compound 1976-85-8 CAS 106-60-5 CAS Protoheme IX farnesyltransferase, mitochondrial HMDB01162 HMDB 1.0 CHEBI:16990 ChEBI 388299 ChemSpider Protoporphyrin IX Uridine diphosphate glucuronic acid O2 Oxygen 31.98983 C49H56FeN4O6 Heme A 852.3549 1.0 Water CHEBI:15436 ChEBI CHEBI:16645 ChEBI CHEBI:15435 ChEBI CHEBI:16526 ChEBI CHEBI:15437 ChEBI 487-90-1 CAS CHEBI:28607 ChEBI HEME_O BioCyc C00500 KEGG Compound Protoporphyrin IX CHEBI:15430 ChEBI 7782-44-7 CAS 1.0 Cytochrome c oxidase assembly protein COX15 homolog CHEBI:15428 ChEBI 1.0 HEME_A BioCyc 9606 TAXONOMY HMDB03325 HMDB 1333-74-0 CAS Coproporphyrinogen-III oxidase, mitochondrial C49H58FeN4O5 Heme O 838.3757 P06132 UniProt Q7KZN9 UniProt CHEBI:17549 ChEBI P53004 UniProt 4573640 ChemSpider HMDB01149 HMDB Uroporphyrin I SMILES OCC1=C(CC(O)=O)C(CCC(O)=O)=C(CC2=C(CC(O)=O)C(CCC(O)=O)=C(CC3=C(CC(O)=O)C(CCC(O)=O)=C(CC4=C(CC(O)=O)C(CCC(O)=O)=CN4)N3)N2)N1 1179 PubChem-compound HMDB01261 HMDB 281 PubChem-compound HMDB00051 HMDB HMDB00295 HMDB 5893 PubChem-compound HMDB00054 HMDB HMDB01022 HMDB HMDB02111 HMDB SMILES CC(C)=CCC\C(C)=C\CC\C(C)=C\COP(O)(=O)OP(O)(O)=O Alcohol CHEBI:30879 ChEBI SMILES NCC(=O)CCC(O)=O SMILES [Fe+3] CHEBI:15377 ChEBI CHEBI:15379 ChEBI 20074-52-6 CAS CHEBI:13193 ChEBI Bilirubin Oxygen Biliverdin reductase A 280 PubChem-compound PROTOPORPHYRIN_IX BioCyc Heme oxygenase 1 HMDB01487 HMDB CHEBI:17407 ChEBI HMDB01008 HMDB 1.0 SMILES CC1=C(CCC(O)=O)/C2=C/C3=N/C(=C\C4=C(C)C(CCC(O)=O)=C(N4)/C=C4\N=C(\C=C\1/N\2)C(CCC(O)=O)=C4C)/C(CCC(O)=O)=C3C SMILES CC1=C2CC3=C(C)C(C=C)=C(CC4=C(C)C(C=C)=C(CC5=C(C)C(CCC(O)=O)=C(CC(N2)=C1CCC(O)=O)N5)N4)N3 HMDB01362 HMDB SMILES CC1=C(C=C)\C(NC1=O)=C/C1=C(C)C(CCC(=O)O[C@@H]2O[C@@H]([C@@H](O)[C@H](O)[C@H]2O)C(O)=O)=C(CC2=C(CCC(=O)O[C@@H]3O[C@@H]([C@@H](O)[C@H](O)[C@H]3O)C(O)=O)C(C)=C(N2)\C=C2\NC(=O)C(C=C)=C2C)N1 HMDB01361 HMDB HMDB02211 HMDB SMILES OC(=O)CCC1=C(CC(O)=O)/C2=C/C3=N/C(=C\C4=C(CCC(O)=O)C(CC(O)=O)=C(N4)/C=C4\N=C(\C=C\1/N\2)C(CC(O)=O)=C4CCC(O)=O)/C(CC(O)=O)=C3CCC(O)=O SMILES O[C@@H]1[C@@H](COP(O)(=O)OP(O)(=O)O[C@H]2O[C@@H]([C@@H](O)[C@H](O)[C@H]2O)C(O)=O)O[C@H]([C@@H]1O)N1C=CC(=O)NC1=O C33H42N4O6 D-Urobilinogen 590.3104 C14819 KEGG Compound 1.0 C14818 KEGG Compound 3-METHYLBENZYLSUCCINYL-COA BioCyc CHEBI:28421 ChEBI HMDB01377 HMDB 5-aminolevulinate synthase, nonspecific, mitochondrial HMDB01137 HMDB 24604415 ChemSpider CHEBI:17659 ChEBI 1.0 2624-63-7 CAS