KEGG   PATHWAY: hsa00860
Entry
hsa00860                    Pathway                                
Name
Porphyrin metabolism - Homo sapiens (human)
Description
Porphyrins are a group of chemical compounds with the backbone ring structure consisting of four linked pyrrole units. Metal complexes of porphyrins, as well as those of corrinoids with the one-carbon shorter ring structure, play important biological roles. They include iron-containing heme, magnesium-containing chlorophyll, nickel-containing coenzyme F430 and cobalt-containing cobamide (such as vitamin B12 coenzyme) as shown in this map.
Class
Metabolism; Metabolism of cofactors and vitamins
Pathway map
hsa00860  Porphyrin metabolism
hsa00860

Module
hsa_M00868  Heme biosynthesis, animals and fungi, glycine => heme [PATH:hsa00860]
Network
  Element
N00601  Heme biosynthesis
Drug
D05922  Stannsoporfin (USAN/INN)
D10003  Hemin (JAN)
D11702  Givosiran (USAN/INN)
D11708  Givosiran sodium (JAN)
Other DBs
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  EPRS1; glutamyl-prolyl-tRNA synthetase 1 [KO:K14163] [EC:6.1.1.17 6.1.1.15]
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 1.3.3.15]
2235  FECH; ferrochelatase [KO:K01772] [EC:4.98.1.1 4.99.1.9]
1352  COX10; cytochrome c oxidase assembly factor heme A:farnesyltransferase COX10 [KO:K02257] [EC:2.5.1.141]
1355  COX15; cytochrome c oxidase assembly homolog COX15 [KO:K02259] [EC:1.17.99.9]
326625  MMAB; metabolism of cobalamin associated B [KO:K00798] [EC:2.5.1.17]
3162  HMOX1; heme oxygenase 1 [KO:K00510] [EC:1.14.14.18]
3163  HMOX2; heme oxygenase 2 [KO:K21418] [EC:1.14.14.18]
644  BLVRA; biliverdin reductase A [KO:K00214] [EC:1.3.1.24]
645  BLVRB; biliverdin reductase B [KO:K05901] [EC:1.3.1.24 1.5.1.30]
10941  UGT2A1; UDP glucuronosyltransferase family 2 member A1 complex locus [KO:K00699] [EC:2.4.1.17]
79799  UGT2A3; UDP glucuronosyltransferase family 2 member A3 [KO:K00699] [EC:2.4.1.17]
7367  UGT2B17; UDP glucuronosyltransferase family 2 member B17 [KO:K00699] [EC:2.4.1.17]
10720  UGT2B11; UDP glucuronosyltransferase family 2 member B11 [KO:K00699] [EC:2.4.1.17]
54490  UGT2B28; UDP glucuronosyltransferase family 2 member B28 [KO:K00699] [EC:2.4.1.17]
54578  UGT1A6; UDP glucuronosyltransferase family 1 member A6 [KO:K00699] [EC:2.4.1.17]
54657  UGT1A4; UDP glucuronosyltransferase family 1 member A4 [KO:K00699] [EC:2.4.1.17]
54658  UGT1A1; UDP glucuronosyltransferase family 1 member A1 [KO:K00699] [EC:2.4.1.17]
54659  UGT1A3; UDP glucuronosyltransferase family 1 member A3 [KO:K00699] [EC:2.4.1.17]
7365  UGT2B10; UDP glucuronosyltransferase family 2 member B10 [KO:K00699] [EC:2.4.1.17]
54600  UGT1A9; UDP glucuronosyltransferase family 1 member A9 [KO:K00699] [EC:2.4.1.17]
7364  UGT2B7; UDP glucuronosyltransferase family 2 member B7 [KO:K00699] [EC:2.4.1.17]
54575  UGT1A10; UDP glucuronosyltransferase family 1 member A10 [KO:K00699] [EC:2.4.1.17]
54576  UGT1A8; UDP glucuronosyltransferase family 1 member A8 [KO:K00699] [EC:2.4.1.17]
54579  UGT1A5; UDP glucuronosyltransferase family 1 member A5 [KO:K00699] [EC:2.4.1.17]
7366  UGT2B15; UDP glucuronosyltransferase family 2 member B15 [KO:K00699] [EC:2.4.1.17]
54577  UGT1A7; UDP glucuronosyltransferase family 1 member A7 [KO:K00699] [EC:2.4.1.17]
7363  UGT2B4; UDP glucuronosyltransferase family 2 member B4 [KO:K00699] [EC:2.4.1.17]
574537  UGT2A2; UDP glucuronosyltransferase family 2 member 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 [KO:K13624] [EC:1.16.3.1]
9843  HEPH; hephaestin [KO:K14735] [EC:1.16.3.1]
341208  HEPHL1; hephaestin like 1 [KO:K14735] [EC:1.16.3.1]
2395  FXN; frataxin [KO:K19054] [EC:1.16.3.1]
2495  FTH1; ferritin heavy chain 1 [KO:K00522] [EC:1.16.3.1]
2512  FTL; ferritin light chain [KO:K13625]
94033  FTMT; ferritin mitochondrial [KO:K18495] [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
C03373  Aminoimidazole ribotide
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  Bacteriopheophytin
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
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  8-Ethyl-12-methyl-3-vinylbacteriochlorophyllide d
C18160  Bacteriochlorophyllide d
C18161  Bacteriochlorophyllide c
C18162  Bacteriochlorophyll d
C18163  Bacteriochlorophyll c
C19608  Ferritin
C20666  5-Oxo-delta-bilirubin
C21217  Geranylgeranyl bacteriochlorophyllide a
C21284  Fe-coproporphyrin III
C21427  8,12-Diethyl-3-vinylbacteriochlorophyllide d
C21428  12-Ethyl-8-propyl-3-vinylbacteriochlorophyllide d
C21429  12-Ethyl-8-isobutyl-3-vinylbacteriochlorophyllide d
C21431  8-Ethyl-12-methylbacteriochlorophyllide d
C21432  8,12-Diethylbacteriochlorophyllide d
C21433  12-Ethyl-8-propylbacteriochlorophyllide d
C21434  12-Ethyl-8-isobutylbacteriochlorophyllide d
C21435  Geranylgeranyl bacteriochlorophyllide b
C21510  Nickel-sirohydrochlorin
C21511  Nickel-sirohydrochlorin a,c-diamide
C21512  15,17(3)-Seco-F430-17(3)-acid
C21582  Bacteriochlorophyllide e
C21723  Biliverdin-IX-delta
C21724  Biliverdin-IX-beta
C21764  5-Hydroxybenzimidazole
C21835  12,18-Didecarboxysiroheme
C22338  Dihydro-heme d1
C22339  Heme d1
C22450  5-Methoxybenzimidazole
C22451  5-Methoxy-6-methylbenzimidazole
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)
DOI:10.1042/bj3300121
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)
DOI:10.1099/13500872-145-3-529
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)
DOI:10.1073/pnas.96.21.11798
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)
DOI:10.1074/jbc.M007146200
Reference
PMID:12196148
  Authors
Scott AI, Roessner CA.
  Title
Biosynthesis of cobalamin (vitamin B(12)).
  Journal
Biochem Soc Trans 30:613-20 (2002)
DOI:10.1042/bst0300613
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)
DOI:10.1039/b108967f
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)
DOI:10.1099/00221287-148-6-1845
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)
DOI:10.1105/tpc.13.4.965
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)
DOI:10.1146/annurev.micro.61.080706.093242
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)
DOI:10.1023/B:PRES.0000004310.96189.b4
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)
DOI:10.1038/nchembio.1086
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)
DOI:10.1073/pnas.1308098110
Related
pathway
hsa00230  Purine metabolism
hsa00250  Alanine, aspartate and glutamate metabolism
hsa00260  Glycine, serine and threonine metabolism
hsa00740  Riboflavin metabolism
KO pathway
ko00860   

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   KEGG NETWORK (1)   
Genome (1)   
   KEGG GENOME (1)   
Gene (46)   
   KEGG GENES (46)   
All databases (48)   

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