KEGG   PATHWAY: hsa00790Help
Entry
hsa00790                    Pathway                                

Name
Folate biosynthesis - Homo sapiens (human)
Class
Metabolism; Metabolism of cofactors and vitamins
BRITE hierarchy
Pathway map
hsa00790  Folate biosynthesis
hsa00790

Ortholog table
Module
hsa_M00842  Tetrahydrobiopterin biosynthesis, GTP => BH4 [PATH:hsa00790]
hsa_M00880  Molybdenum cofactor biosynthesis, GTP => molybdenum cofactor [PATH:hsa00790]
Network
nt06016  Phenylalanine and tyrosine metabolism
nt06025  Molybdenum cofactor biosynthesis
  Element
N00702  Tetrahydrobiopterin biosynthesis, GTP to BH4
N00703  PTS deficiency in tetrahydrobiopterin biosynthesis
N00704  GCH1 deficiency in tetrahydrobiopterin biosynthesis
N00705  Tetrahydrobiopterin biosynthesis, BH4OH to BH4
N00706  QDPR deficiency in tetrahydrobiopterin biosynthesis
N00707  PCBD1 deficiency in tetrahydrobiopterin biosynthesis
N00890  Molybdenum cofactor biosynthesis
N00891  MOCOS deficiency in molybdenum cofactor biosynthesis
N00892  GPHN deficiency in molybdenum cofactor biosynthesis
N00893  MOCS2 deficiency in molybdenum cofactor biosynthesis
N00894  MOCS1 deficiency in molybdenum cofactor biosynthesis
Disease
H00167  Phenylketonuria
H00213  Hypophosphatasia
H00831  Primary dystonia
H01197  Dihydrofolate reductase deficiency
H02311  Molybdenum cofactor deficiency
Drug
D00142  Methotrexate (JP17/USP/INN)
D02115  Methotrexate sodium
D03828  Pemetrexed disodium (USAN)
D03942  Edatrexate (USAN/INN)
D05500  Piritrexim isethionate (USAN)
D05589  Pralatrexate (JAN/USAN/INN)
D05999  Talotrexin ammonium (USAN)
D06238  Trimetrexate (USAN/INN)
D06239  Trimetrexate glucuronate (USAN)
D06503  Pemetrexed sodium hydrate (JAN)
D07472  Pemetrexed (INN)
D10596  Pemetrexed sodium hemipentahydrate (JAN)
D11352  Pemetrexed tromethamine (USAN)
Other DBs
GO: 0046656
Organism
Homo sapiens (human) [GN:hsa]
Gene
2643  GCH1; GTP cyclohydrolase 1 [KO:K01495] [EC:3.5.4.16]
249  ALPL; alkaline phosphatase, biomineralization associated [KO:K01077] [EC:3.1.3.1]
248  ALPI; alkaline phosphatase, intestinal [KO:K01077] [EC:3.1.3.1]
250  ALPP; alkaline phosphatase, placental [KO:K01077] [EC:3.1.3.1]
251  ALPG; alkaline phosphatase, germ cell [KO:K01077] [EC:3.1.3.1]
1719  DHFR; dihydrofolate reductase [KO:K00287] [EC:1.5.1.3]
200895  DHFR2; dihydrofolate reductase 2 [KO:K00287] [EC:1.5.1.3]
2356  FPGS; folylpolyglutamate synthase [KO:K01930] [EC:6.3.2.17]
8836  GGH; gamma-glutamyl hydrolase [KO:K01307] [EC:3.4.19.9]
5805  PTS; 6-pyruvoyltetrahydropterin synthase [KO:K01737] [EC:4.1.2.50 4.2.3.12]
6697  SPR; sepiapterin reductase [KO:K00072] [EC:1.1.1.153]
231  AKR1B1; aldo-keto reductase family 1 member B [KO:K00011] [EC:1.1.1.21]
57016  AKR1B10; aldo-keto reductase family 1 member B10 [KO:K00011] [EC:1.1.1.21]
873  CBR1; carbonyl reductase 1 [KO:K00079] [EC:1.1.1.197 1.1.1.189 1.1.1.184]
8644  AKR1C3; aldo-keto reductase family 1 member C3 [KO:K04119] [EC:1.1.1.357 1.1.1.213 1.1.1.188 1.1.1.51]
5860  QDPR; quinoid dihydropteridine reductase [KO:K00357] [EC:1.5.1.34]
84105  PCBD2; pterin-4 alpha-carbinolamine dehydratase 2 [KO:K01724] [EC:4.2.1.96]
5092  PCBD1; pterin-4 alpha-carbinolamine dehydratase 1 [KO:K01724] [EC:4.2.1.96]
5053  PAH; phenylalanine hydroxylase [KO:K00500] [EC:1.14.16.1]
7054  TH; tyrosine hydroxylase [KO:K00501] [EC:1.14.16.2]
121278  TPH2; tryptophan hydroxylase 2 [KO:K00502] [EC:1.14.16.4]
7166  TPH1; tryptophan hydroxylase 1 [KO:K00502] [EC:1.14.16.4]
4337  MOCS1; molybdenum cofactor synthesis 1 [KO:K20967] [EC:4.6.1.17 4.1.99.22]
4338  MOCS2; molybdenum cofactor synthesis 2 [KO:K03635] [EC:2.8.1.12]
10243  GPHN; gephyrin [KO:K15376] [EC:2.7.7.75 2.10.1.1]
55034  MOCOS; molybdenum cofactor sulfurase [KO:K15631] [EC:2.8.1.9]
Compound
C00044  GTP
C00101  Tetrahydrofolate
C00119  5-Phospho-alpha-D-ribose 1-diphosphate
C00156  4-Hydroxybenzoate
C00251  Chorismate
C00266  Glycolaldehyde
C00268  Dihydrobiopterin
C00272  Tetrahydrobiopterin
C00415  Dihydrofolate
C00504  Folate
C00568  4-Aminobenzoate
C00835  Sepiapterin
C00921  Dihydropteroate
C01217  5,6,7,8-Tetrahydromethanopterin
C01300  6-(Hydroxymethyl)-7,8-dihydropterin
C01304  2,5-Diamino-6-(5-phospho-D-ribosylamino)pyrimidin-4(3H)-one
C02587  Pyrimidodiazepine
C02953  7,8-Dihydrobiopterin
C03541  THF-polyglutamate
C03684  6-Pyruvoyltetrahydropterin
C04244  6-Lactoyl-5,6,7,8-tetrahydropterin
C04807  6-Hydroxymethyl-7,8-dihydropterin diphosphate
C04874  7,8-Dihydroneopterin
C04895  7,8-Dihydroneopterin 3'-triphosphate
C05922  Formamidopyrimidine nucleoside triphosphate
C05923  2,5-Diaminopyrimidine nucleoside triphosphate
C05924  Molybdopterin
C05925  Dihydroneopterin phosphate
C05926  Neopterin
C05927  7,8-Dihydromethanopterin
C06148  2,5-Diamino-6-(5'-triphosphoryl-3',4'-trihydroxy-2'-oxopentyl)-amino-4-oxopyrimidine
C09332  THF-L-glutamate
C11355  4-Amino-4-deoxychorismate
C15522  4a-Hydroxytetrahydrobiopterin
C15996  7-Cyano-7-carbaguanine
C16675  7-Aminomethyl-7-carbaguanine
C18237  Molybdoenzyme molybdenum cofactor
C18239  Precursor Z
C18802  Tetrahydrosarcinapterin
C19848  Adenylated molybdopterin
C19871  Guanylyl molybdenum cofactor
C20239  6-Carboxy-5,6,7,8-tetrahydropterin
C20248  7-Carboxy-7-carbaguanine
C20264  L-threo-Tetrahydrobiopterin
C20559  4-(beta-D-Ribofuranosyl)aniline 5'-phosphate
C20562  N-[(7,8-Dihydropterin-6-yl)methyl]-4-(beta-D-ribofuranosyl)aniline 5'-phosphate
C20566  7,8-Dihydroneopterin 2'-phosphate
C20567  7,8-Dihydroneopterin 2',3'-cyclic phosphate
C21007  5,6,7,8-Tetrahydromonapterin
C21008  7,8-Dihydromonapterin
C21065  7,8-Dihydroxanthopterin
C21094  7,8-Dihydromonapterin 3'-triphosphate
C21107  4-(beta-D-Ribofuranosyl)phenol 5'-phosphate
C21310  (8S)-3',8-Cyclo-7,8-dihydroguanosine 5'-triphosphate
C21485  Cytidylyl molybdenum cofactor
C21486  Thio-molybdenum cofactor
C21640  6-(1'-Hydroxy-2'-oxopropyl)-tetrahydropterin
Reference
  Authors
Nishizuka Y, Seyama Y, Ikai A, Ishimura Y, Kawaguchi A (eds).
  Title
[Cellular Functions and Metabolic Maps] (In Japanese)
  Journal
Tokyo Kagaku Dojin (1997)
Reference
  Authors
Schwarz G, Mendel RR, Ribbe MW
  Title
Molybdenum cofactors, enzymes and pathways.
  Journal
Nature 460:839-47 (2009)
DOI:10.1038/nature08302
Reference
  Authors
Hanzelmann P, Schindelin H
  Title
Binding of 5'-GTP to the C-terminal FeS cluster of the radical S-adenosylmethionine enzyme MoaA provides insights into its mechanism.
  Journal
Proc Natl Acad Sci U S A 103:6829-34 (2006)
DOI:10.1073/pnas.0510711103
Reference
  Authors
Daniels JN, Wuebbens MM, Rajagopalan KV, Schindelin H
  Title
Crystal structure of a molybdopterin synthase-precursor Z complex: insight into its sulfur transfer mechanism and its role in molybdenum cofactor deficiency.
  Journal
Biochemistry 47:615-26 (2008)
DOI:10.1021/bi701734g
Reference
  Authors
Wuebbens MM, Rajagopalan KV
  Title
Mechanistic and mutational studies of Escherichia coli molybdopterin synthase clarify the final step of molybdopterin biosynthesis.
  Journal
J Biol Chem 278:14523-32 (2003)
DOI:10.1074/jbc.M300453200
Reference
  Authors
Werner ER, Blau N, Thony B
  Title
Tetrahydrobiopterin: biochemistry and pathophysiology.
  Journal
Biochem J 438:397-414 (2011)
DOI:10.1042/BJ20110293
Reference
  Authors
Iino T, Tabata M, Takikawa S, Sawada H, Shintaku H, Ishikura S, Hara A
  Title
Tetrahydrobiopterin is synthesized from 6-pyruvoyl-tetrahydropterin by the human aldo-keto reductase AKR1 family members.
  Journal
Arch Biochem Biophys 416:180-7 (2003)
DOI:10.1016/S0003-9861(03)00295-9
Reference
PMID:1902669
  Authors
Park YS, Heizmann CW, Wermuth B, Levine RA, Steinerstauch P, Guzman J, Blau N
  Title
Human carbonyl and aldose reductases: new catalytic functions in tetrahydrobiopterin biosynthesis.
  Journal
Biochem Biophys Res Commun 175:738-44 (1991)
DOI:10.1016/0006-291X(91)91628-P
KO pathway
ko00790   

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