ENTRY hsa00860 Pathway NAME Porphyrin and chlorophyll metabolism - Homo sapiens (human) CLASS Metabolism; Metabolism of cofactors and vitamins PATHWAY_MAP hsa00860 Porphyrin and chlorophyll metabolism DISEASE H00201 Erythropoietic porphyria (EP) H00202 Hepatic porphyria H00208 Hyperbilirubinemia H00833 Neurodegeneration with brain iron accumulation (NBIA) H01027 Anophthalmia and microphthalmia (A/M) H01202 Cataract H01368 Cytochrome c oxidase (COX) deficiency DBLINKS BSID: 83021 GO: 0006778 ORGANISM Homo sapiens (human) [GN:hsa] GENE 211 ALAS1; 5'-aminolevulinate synthase 1 [KO:K00643] [EC:2.3.1.37] 212 ALAS2; 5'-aminolevulinate synthase 2 [KO:K00643] [EC:2.3.1.37] 124454 EARS2; glutamyl-tRNA synthetase 2, mitochondrial [KO:K01885] [EC:6.1.1.17] 2058 EPRS; glutamyl-prolyl-tRNA synthetase [KO:K14163] [EC:6.1.1.15 6.1.1.17] 210 ALAD; aminolevulinate dehydratase [KO:K01698] [EC:4.2.1.24] 3145 HMBS; hydroxymethylbilane synthase [KO:K01749] [EC:2.5.1.61] 7390 UROS; uroporphyrinogen III synthase [KO:K01719] [EC:4.2.1.75] 7389 UROD; uroporphyrinogen decarboxylase [KO:K01599] [EC:4.1.1.37] 1371 CPOX; coproporphyrinogen oxidase [KO:K00228] [EC:1.3.3.3] 5498 PPOX; protoporphyrinogen oxidase [KO:K00231] [EC:1.3.3.4] 2235 FECH; ferrochelatase [KO:K01772] [EC:4.99.1.1] 1352 COX10; COX10 heme A:farnesyltransferase cytochrome c oxidase assembly factor [KO:K02257] [EC:2.5.1.-] 1355 COX15; cytochrome c oxidase assembly homolog 15 (yeast) [KO:K02259] 326625 MMAB; methylmalonic aciduria (cobalamin deficiency) cblB type [KO:K00798] [EC:2.5.1.17] 3162 HMOX1; heme oxygenase (decycling) 1 [KO:K00510] [EC:1.14.99.3] 3163 HMOX2; heme oxygenase (decycling) 2 [KO:K00510] [EC:1.14.99.3] 644 BLVRA; biliverdin reductase A [KO:K00214] [EC:1.3.1.24] 645 BLVRB; biliverdin reductase B [KO:K05901] [EC:1.5.1.30 1.3.1.24] 10941 UGT2A1; UDP glucuronosyltransferase 2 family, polypeptide A1, complex locus [KO:K00699] [EC:2.4.1.17] 79799 UGT2A3; UDP glucuronosyltransferase 2 family, polypeptide A3 [KO:K00699] [EC:2.4.1.17] 7367 UGT2B17; UDP glucuronosyltransferase 2 family, polypeptide B17 [KO:K00699] [EC:2.4.1.17] 10720 UGT2B11; UDP glucuronosyltransferase 2 family, polypeptide B11 [KO:K00699] [EC:2.4.1.17] 54490 UGT2B28; UDP glucuronosyltransferase 2 family, polypeptide B28 [KO:K00699] [EC:2.4.1.17] 54578 UGT1A6; UDP glucuronosyltransferase 1 family, polypeptide A6 [KO:K00699] [EC:2.4.1.17] 54657 UGT1A4; UDP glucuronosyltransferase 1 family, polypeptide A4 [KO:K00699] [EC:2.4.1.17] 54658 UGT1A1; UDP glucuronosyltransferase 1 family, polypeptide A1 [KO:K00699] [EC:2.4.1.17] 54659 UGT1A3; UDP glucuronosyltransferase 1 family, polypeptide A3 [KO:K00699] [EC:2.4.1.17] 7365 UGT2B10; UDP glucuronosyltransferase 2 family, polypeptide B10 [KO:K00699] [EC:2.4.1.17] 54600 UGT1A9; UDP glucuronosyltransferase 1 family, polypeptide A9 [KO:K00699] [EC:2.4.1.17] 7364 UGT2B7; UDP glucuronosyltransferase 2 family, polypeptide B7 [KO:K00699] [EC:2.4.1.17] 54575 UGT1A10; UDP glucuronosyltransferase 1 family, polypeptide A10 [KO:K00699] [EC:2.4.1.17] 54576 UGT1A8; UDP glucuronosyltransferase 1 family, polypeptide A8 [KO:K00699] [EC:2.4.1.17] 54579 UGT1A5; UDP glucuronosyltransferase 1 family, polypeptide A5 [KO:K00699] [EC:2.4.1.17] 7366 UGT2B15; UDP glucuronosyltransferase 2 family, polypeptide B15 [KO:K00699] [EC:2.4.1.17] 54577 UGT1A7; UDP glucuronosyltransferase 1 family, polypeptide A7 [KO:K00699] [EC:2.4.1.17] 7363 UGT2B4; UDP glucuronosyltransferase 2 family, polypeptide B4 [KO:K00699] [EC:2.4.1.17] 574537 UGT2A2; UDP glucuronosyltransferase 2 family, polypeptide A2 [KO:K00699] [EC:2.4.1.17] 2990 GUSB; glucuronidase, beta [KO:K01195] [EC:3.2.1.31] 3052 HCCS; holocytochrome c synthase [KO:K01764] [EC:4.4.1.17] 1356 CP; ceruloplasmin (ferroxidase) [KO:K13624] [EC:1.16.3.1] 9843 HEPH; hephaestin [KO:K14735] [EC:1.16.3.1] 2395 FXN; frataxin [KO:K19054] [EC:1.16.3.1] COMPOUND C00025 L-Glutamate C00032 Heme C00037 Glycine C00188 L-Threonine C00194 Cobamide coenzyme C00430 5-Aminolevulinate C00486 Bilirubin C00500 Biliverdin C00524 Cytochrome c C00541 Cob(II)alamin C00748 Siroheme C00853 Cob(I)alamin C00931 Porphobilinogen C00992 Aquacob(III)alamin C01024 Hydroxymethylbilane C01051 Uroporphyrinogen III C01079 Protoporphyrinogen IX C01708 Hemoglobin C02139 Chlorophyllide C02191 Protoporphyrin C02463 Precorrin 2 C02469 Uroporphyrin III C02800 4,5-Dioxopentanoate C02823 Cyanocobalamin C02880 Protochlorophyllide C02987 L-Glutamyl-tRNA(Glu) C03029 Transferrin[Fe(II)]2 C03114 Dimethylbenzimidazole C03179 Transferrin[Fe(III)]2 C03194 (R)-1-Aminopropan-2-ol C03263 Coproporphyrinogen III C03516 Magnesium protoporphyrin C03741 (S)-4-Amino-5-oxopentanoate C04122 D-1-Aminopropan-2-ol O-phosphate C04536 Magnesium protoporphyrin monomethyl ester C04778 N1-(5-Phospho-alpha-D-ribosyl)-5,6-dimethylbenzimidazole C05306 Chlorophyll a C05307 Chlorophyll b C05766 Uroporphyrinogen I C05767 Uroporphyrin I C05768 Coproporphyrinogen I C05769 Coproporphyrin I C05770 Coproporphyrin III C05772 Precorrin 3A C05773 Cobyrinate C05774 Cobinamide C05775 alpha-Ribazole C05777 Coenzyme F430 C05778 Sirohydrochlorin C05779 Apoferritin C05780 Apotransferrin C05781 Oxyhemoglobin C05782 Myoglobin C05783 Cytochrome a C05784 Catalase C05785 Peroxidase C05786 (3Z)-Phycocyanobilin C05787 Bilirubin beta-diglucuronide C05789 L-Urobilinogen C05790 I-Urobilinogen C05791 D-Urobilinogen C05793 L-Urobilin C05794 Urobilin C05795 D-Urobilin C05797 Pheophytin a C05798 Bacterio-pheophytins C05912 (3Z)-Phycoerythrobilin C05913 (3Z)-Phytochromobilin C06319 Precorrin 6Y C06320 Precorrin 6X C06399 Hydrogenobyrinate C06406 Precorrin 3B C06407 Precorrin 4 C06408 Precorrin 8X C06416 Precorrin 5 C06503 Hydrogenobyrinate a,c diamide C06504 Cob(II)yrinate a,c diamide C06505 Cob(I)yrinate a,c diamide C06506 Adenosyl cobyrinate a,c diamide C06507 Adenosyl cobyrinate hexaamide C06508 Adenosyl cobinamide C06509 Adenosyl cobinamide phosphate C06510 Adenosine-GDP-cobinamide C11242 Bacteriochlorophyll a C11243 Bacteriochlorophyll b C11538 Cobalt-sirohydrochlorin C11539 Cobalt-precorrin 3 C11540 Cobalt-precorrin 4 C11542 Cobalt-precorrin 6 C11543 Cobalt-dihydro-precorrin 6 C11545 Cobalt-precorrin 8 C11630 15,16-Dihydrobiliverdin C11829 13(1)-Hydroxy-magnesium-protoporphyrin IX 13-monomethyl ester C11830 13(1)-Oxo-magnesium-protoporphyrin IX 13-monomethyl ester C11831 Divinylprotochlorophyllide C11832 Divinyl chlorophyllide a C11850 Divinylchlorophyll a C11851 Zn-Bacteriochlorophyll a C12147 L-Threonine O-3-phosphate C14818 Fe2+ C14819 Fe3+ C15670 Heme A C15672 Heme O C16242 Cobalt-precorrin 5A C16243 Cobalt-precorrin 5B C16244 Cobalt-precorrin 7 C16540 7(1)-Hydroxychlorophyllide a C16541 Chlorophyllide b C17401 Cobalt-factor III C18021 Pheophorbide a C18022 Red chlorophyll catabolite C18064 Pyropheophorbide a C18098 Primary fluorescent chlorophyll catabolite C18151 7(1)-Hydroxychlorophyll a C18152 3-Vinylbacteriochlorophyllide a C18153 3-Hydroxyethylbacteriochlorophyllide a C18154 3-Hydroxyethylchlorophyllide a C18155 Bacteriochlorophyllide a C18156 Bacteriochlorophyllide b C18157 3-Vinylbacteriochlorophyllide d C18160 Bacteriochlorophyllide d C18161 Bacteriochlorophyllide c C18162 Bacteriochlorophyll d C18163 Bacteriochlorophyll c REFERENCE PMID:9461500 AUTHORS Woodcock SC, Raux E, Levillayer F, Thermes C, Rambach A, Warren MJ. TITLE Effect of mutations in the transmethylase and dehydrogenase/chelatase domains of sirohaem synthase (CysG) on sirohaem and cobalamin biosynthesis. JOURNAL Biochem J 330:121-9 (1998) REFERENCE PMID:10217486 AUTHORS Johansson P, Hederstedt L TITLE Organization of genes for tetrapyrrole biosynthesis in gram--positive bacteria. JOURNAL Microbiology 145 ( Pt 3):529-38 (1999) REFERENCE PMID:10518530 AUTHORS Maggio-Hall LA, Escalante-Semerena JC. TITLE In vitro synthesis of the nucleotide loop of cobalamin by Salmonella typhimurium enzymes. JOURNAL Proc Natl Acad Sci USA 96:11798-803 (1999) REFERENCE PMID:11007789 AUTHORS Roper JM, Raux E, Brindley AA, Schubert HL, Gharbia SE, Shah HN, Warren MJ. TITLE The enigma of cobalamin (Vitamin B12) biosynthesis in Porphyromonas gingivalis. Identification and characterization of a functional corrin pathway. JOURNAL J Biol Chem 275:40316-23 (2000) REFERENCE PMID:12196148 AUTHORS Scott AI, Roessner CA. TITLE Biosynthesis of cobalamin (vitamin B(12)). JOURNAL Biochem Soc Trans 30:613-20 (2002) REFERENCE PMID:12195810 AUTHORS Warren MJ, Raux E, Schubert HL, Escalante-Semerena JC. TITLE The biosynthesis of adenosylcobalamin (vitamin B12). JOURNAL Nat Prod Rep 19:390-412 (2002) REFERENCE PMID:12055304 AUTHORS Roessner CA, Huang KX, Warren MJ, Raux E, Scott AI. TITLE Isolation and characterization of 14 additional genes specifying the anaerobic biosynthesis of cobalamin (vitamin B12) in Propionibacterium freudenreichii (P. shermanii). JOURNAL Microbiology 148:1845-53 (2002) REFERENCE PMID:11283349 AUTHORS Frankenberg N, Mukougawa K, Kohchi T, Lagarias JC. TITLE Functional genomic analysis of the HY2 family of ferredoxin-dependent bilin reductases from oxygenic photosynthetic organisms. JOURNAL Plant Cell 13:965-78 (2001) REFERENCE PMID:17506685 AUTHORS Chew AG, Bryant DA TITLE Chlorophyll biosynthesis in bacteria: the origins of structural and functional diversity. JOURNAL Annu Rev Microbiol 61:113-29 (2007) REFERENCE PMID:16245042 AUTHORS Frigaard NU, Chew AG, Li H, Maresca JA, Bryant DA TITLE Chlorobium tepidum: insights into the structure, physiology, and metabolism of a green sulfur bacterium derived from the complete genome sequence. JOURNAL Photosynth Res 78:93-117 (2003) REFERENCE PMID:23042036 AUTHORS Deery E, Schroeder S, Lawrence AD, Taylor SL, Seyedarabi A, Waterman J, Wilson KS, Brown D, Geeves MA, Howard MJ, Pickersgill RW, Warren MJ TITLE An enzyme-trap approach allows isolation of intermediates in cobalamin biosynthesis. JOURNAL Nat Chem Biol 8:933-40 (2012) REFERENCE PMID:23922391 AUTHORS Moore SJ, Lawrence AD, Biedendieck R, Deery E, Frank S, Howard MJ, Rigby SE, Warren MJ TITLE Elucidation of the anaerobic pathway for the corrin component of cobalamin (vitamin B12). JOURNAL Proc Natl Acad Sci U S A 110:14906-11 (2013) KO_PATHWAY ko00860 ///