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P09601 UniProt 7722-84-1 CAS 439161 PubChem-compound C00430 KEGG Compound 937 ChemSpider Transport1219 false Heme (→) Transport: Homo sapiens, Cell, Mitochondrion to Homo sapiens, Cell, Cytosol LEFT_TO_RIGHT Porphobilinogen deaminase CHEBI:16134 ChEBI UROPORPHYRIN_III BioCyc COPROPORPHYRINOGEN_I BioCyc 29936 PubChem-compound HMDB0003125 HMDB HMDB0002158 HMDB 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 Heme 1.0 1146 ChemSpider CHEBI:15380 ChEBI NADH C25H40N7O19P3S Succinyl-CoA 867.1312 CHEBI:17200 ChEBI 952 ChemSpider ALPHA-GLUCOSE-16-BISPHOSPHATE BioCyc HMDB0001086 HMDB UDP-GLUCURONATE BioCyc 6031 PubChem-compound Transport1220 false Protoporphyrin IX (←) Transport: Homo sapiens, Cell, Mitochondrial Inner Membrane to Homo sapiens, Cell, Mitochondrial Matrix LEFT_TO_RIGHT PW000157 PathWhiz 1.0 CHEBI:16240 ChEBI 1.0 445713 PubChem-compound P10746 UniProt 1.0 27815 ChemSpider P22830 UniProt Fe2+ Uridine 5'-diphosphate 531-14-6 CAS CHEBI:27484 ChEBI HMDB0001097 HMDB 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 1.0 BILIRUBIN BioCyc 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 HMDB0003178 HMDB 1.0 Transport178 false Coproporphyrinogen III (→) Transport: Homo sapiens, Cell, Cytosol to Homo sapiens, Cell, Mitochondrion LEFT_TO_RIGHT C21H28N7O14P2 NAD 664.11694 C00011 KEGG Compound HMDB0000902 HMDB 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 HMDB0004158 HMDB 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 Protoporphyrinogen IX 53-84-9 CAS O75310 UniProt HMDB0000916 HMDB C00027 KEGG Compound 553-12-8 CAS 1.0 1.0 Uroporphyrinogen I 3.0 Fe Fe2+ 55.93494 ReactionCatalysis1717 ACTIVATION 73023-76-4 CAS 372-97-4 CAS ReactionCatalysis1716 ACTIVATION 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 Hydrogen peroxide 4.0 Beta-glucuronidase C00282 KEGG Compound 4.0 CHEBI:18392 ChEBI ReactionCatalysis1726 ACTIVATION P50336 UniProt HYDROGEN-PEROXIDE BioCyc AH2 C33H34N4O6 Biliverdin 582.24786 4.0 C02469 KEGG Compound 767 ChemSpider C00167 KEGG Compound Alcohol 1.0 763 ChemSpider 604-98-8 CAS 762 ChemSpider C9H14N2O12P2 Uridine 5'-diphosphate 404.0022 C15H28O7P2 Farnesyl pyrophosphate 382.131 784 PubChem-compound C40H46N4O17 Hydroxymethylbilane 854.28577 C15672 KEGG Compound 1.0 788 PubChem-compound C15670 KEGG Compound C01024 KEGG Compound Uroporphyrinogen-III synthase Heme A 7732-18-5 CAS BILIRUBIN-BISGLUCURONOSIDE BioCyc Coproporphyrin I 315 ChemSpider HMDB0001487 HMDB ReactionCatalysis1741 ACTIVATION PROTOPORPHYRINOGEN BioCyc 17208-65-0 CAS AMMONIA BioCyc SMILES N HMDB0001008 HMDB C5H9NO3 5-Aminolevulinic acid 131.05824 CHEBI:29033 ChEBI Heme HMDB0001362 HMDB HMDB0002211 HMDB CHEBI:29034 ChEBI HMDB0001361 HMDB 16522 ChemSpider Fe Fe3+ 55.93494 Porphobilinogen 5353439 PubChem-compound NAD 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 HMDB0001377 HMDB Ferritin, mitochondrial Coproporphyrinogen III HMDB0001137 HMDB 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 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 HMDB0003325 HMDB 730 ChemSpider SMILES O=O HMDB0001149 HMDB 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 HMDB0000051 HMDB HMDB0001261 HMDB HMDB0002111 HMDB HMDB0000295 HMDB 5809 ChemSpider HMDB0001022 HMDB HMDB0000054 HMDB 1.0 Ferrochelatase, mitochondrial Delta-aminolevulinic acid dehydratase C00091 KEGG Compound 4.0 C36H44N4O8 Coproporphyrinogen I 660.3159 Protoporphyrinogen oxidase 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 Reaction1911 false Heme O → Heme A LEFT_TO_RIGHT HMDB0001162 HMDB 1867-62-5 CAS NADH BioCyc NAD BioCyc 15438-31-0 CAS Farnesyl pyrophosphate 1.0 1.0 1.0 UROPORPHYRIN_I BioCyc 977 PubChem-compound 31110-56-2 CAS 25394 ChemSpider 1.0 ReactionCatalysis1706 ACTIVATION C01079 KEGG Compound 2.0 ReactionCatalysis1708 ACTIVATION ReactionCatalysis1709 ACTIVATION 5-AMINO-LEVULINATE BioCyc C03263 KEGG Compound ReactionCatalysis1710 ACTIVATION 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 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 Protoporphyrin IX 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 2.0 C34H34N4O4 Protoporphyrin IX 562.258 16736701 ChemSpider SMILES [Fe++] GO:0005829 GENE ONTOLOGY 275 ChemSpider 274 ChemSpider 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 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 Oxygen 124-38-9 CAS C2H5NO2 Glycine 75.03203 Fe2+ HMDB0000570 HMDB HMDB0000692 HMDB 4.0 3.0 1.0 1.0 635-65-4 CAS 1.0 Q8N4E7 UniProt Heme O 5257127 PubChem-compound GO:0005759 GENE ONTOLOGY 17473 PubChem-compound 7664-41-7 CAS 1.0 Glycine and Serine Metabolism SubPathway 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 C21H29N7O14P2 NADH 665.12476 1.0 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 HMDB0000123 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 HMDB0000245 HMDB GO:0005739 GENE ONTOLOGY HMDB0006901 HMDB 3.0 PORPHOBILINOGEN BioCyc Biliverdin HMDB0000241 HMDB Heme O CHEBI:17627 ChEBI P08236 UniProt 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 5682 ChemSpider UDP BioCyc 440784 PubChem-compound 1.3.3.4 false 1.3.3.4 3 Oxygen + Protoporphyrinogen IX → 3 Hydrogen peroxide + Protoporphyrin IX LEFT_TO_RIGHT CHEBI:28766 ChEBI 14643-66-4 CAS 14875-96-8 CAS CHEBI:15439 ChEBI SMP0000004 SMPDB 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(CCC(O)=O)=C4CC(O)=O)/C(CC(O)=O)=C3CCC(O)=O HMDB0000936 HMDB 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 HMDB0000935 HMDB 440776 PubChem-compound 440775 PubChem-compound Carbon dioxide 4.1.1.37 false 4.1.1.37 Uroporphyrinogen I → 4 Carbon dioxide + Coproporphyrinogen I LEFT_TO_RIGHT 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 Reaction1883 false Coproporphyrinogen I → Coproporphyrin I LEFT_TO_RIGHT 5459911 PubChem-compound 3.0 1976-85-8 CAS Homo sapiens 106-60-5 CAS Protoheme IX farnesyltransferase, mitochondrial CHEBI:16990 ChEBI 388299 ChemSpider Uridine diphosphate glucuronic acid Protoporphyrin IX O2 Oxygen 31.98983 C49H56FeN4O6 Heme A 852.3549 1.0 Water CHEBI:15436 ChEBI CHEBI:16645 ChEBI CHEBI:15435 ChEBI HMDB0012943 HMDB 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 HMDB0000961 HMDB Cytochrome c oxidase assembly protein COX15 homolog CHEBI:15428 ChEBI 1.0 HEME_A BioCyc Acute Intermittent Porphyria 9606 TAXONOMY 1333-74-0 CAS Coproporphyrinogen-III oxidase, mitochondrial C49H58FeN4O5 Heme O 838.3757 P06132 UniProt Q7KZN9 UniProt CHEBI:17549 ChEBI P53004 UniProt 4573640 ChemSpider 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 281 PubChem-compound 5893 PubChem-compound 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 3.0 SMILES [Fe+3] 1.0 CHEBI:15377 ChEBI CHEBI:15379 ChEBI Ferrochelatase, mitochondrial 20074-52-6 CAS CHEBI:13193 ChEBI Bilirubin Oxygen Biliverdin reductase A 280 PubChem-compound PROTOPORPHYRIN_IX BioCyc Heme oxygenase 1 CHEBI:17407 ChEBI 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 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 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 SMP0000344 SMPDB 3-METHYLBENZYLSUCCINYL-COA BioCyc CHEBI:28421 ChEBI 5-aminolevulinate synthase, nonspecific, mitochondrial 24604415 ChemSpider CHEBI:17659 ChEBI HMDB0001967 HMDB 1.0 2624-63-7 CAS HMDB0000643 HMDB