KEGG   PATHWAY: hsa00900Help
Entry
hsa00900                    Pathway                                

Name
Terpenoid backbone biosynthesis - Homo sapiens (human)
Description
Terpenoids, also known as isoprenoids, are a large class of natural products consisting of isoprene (C5) units. There are two biosynthetic pathways, the mevalonate pathway [MD:M00095] and the non-mevalonate pathway or the MEP/DOXP pathway [MD:M00096], for the terpenoid building blocks: isopentenyl diphosphate (IPP) and dimethylallyl diphosphate (DMAPP). The action of prenyltransferases then generates higher-order building blocks: geranyl diphosphate (GPP), farsenyl diphosphate (FPP), and geranylgeranyl diphosphate (GGPP), which are the precursors of monoterpenoids (C10), sesquiterpenoids (C15), and diterpenoids (C20), respectively. Condensation of these building blocks gives rise to the precursors of sterols (C30) and carotenoids (C40). The MEP/DOXP pathway is absent in higher animals and fungi, but in green plants the MEP/DOXP and mevalonate pathways co-exist in separate cellular compartments. The MEP/DOXP pathway, operating in the plastids, is responsible for the formation of essential oil monoterpenes and linalyl acetate, some sesquiterpenes, diterpenes, and carotenoids and phytol. The mevalonate pathway, operating in the cytosol, gives rise to triterpenes, sterols, and most sesquiterpenes.
Class
Metabolism; Metabolism of terpenoids and polyketides
BRITE hierarchy
Pathway map
hsa00900  Terpenoid backbone biosynthesis
hsa00900

Ortholog table
Module
hsa_M00095  C5 isoprenoid biosynthesis, mevalonate pathway [PATH:hsa00900]
hsa_M00367  C10-C20 isoprenoid biosynthesis, non-plant eukaryotes [PATH:hsa00900]
Disease
H00206  Mevalonate kinase deficiency
H00527  Retinitis pigmentosa (RP)
H00663  Restrictive dermopathy
H00999  Coenzyme Q10 deficiency
H01123  HMG-CoA synthase (HMGCS) deficiency
H01933  Porokeratosis
Drug
D00359  Lovastatin (USP/INN)
D00434  Simvastatin (JP17/USP/INN)
D00887  Atorvastatin calcium anhydorous
D00889  Cerivastatin sodium (JAN/USAN)
D00892  Fluvastatin sodium (JAN/USAN)
D00893  Pravastatin sodium (JP17/USAN)
D00939  Alendronate sodium (USAN)
D00941  Pamidronate disodium (USAN)
D00942  Risedronate sodium (USP)
D01862  Pitavastatin calcium (JAN)
D01915  Rosuvastatin calcium (JAN/USAN)
D01968  Zoledronic acid (USAN)
D02258  Atorvastatin calcium (USP)
D03234  Sodium risedronate hydrate (JP17)
D03601  Crilvastatin (USAN/INN)
D03643  Dalvastatin (USAN/INN)
D03720  Tipifarnib (USAN/INN)
D03816  Tenivastatin calcium (USAN)
D04486  Ibandronate sodium (USAN)
D04768  Lonafarnib (USAN/INN)
D06378  Zoledronate disodium (USAN)
D06379  Zoledronate trisodium (USAN)
D07119  Alendronic acid (INN)
D07123  Incadronate disodium hydrate (JAN)
D07281  Pamidronic acid (INN)
D07474  Atorvastatin (INN)
D07661  Cerivastatin (INN)
D07983  Fluvastatin (INN)
D08056  Ibandronic acid (INN)
D08073  Incadronic acid (INN)
D08410  Pravastatin (INN)
D08484  Risedronic acid (INN)
D08492  Rosuvastatin (INN)
D08689  Zoledronic acid (INN)
D09198  Minodronic acid hydrate (JAN)
D10515  Zoledronic acid hemipentahydrate (JAN)
D10568  Pitavastatin calcium hydrate (JP17)
Other DBs
BSID: 83022
GO: 0009240 0016091
Organism
Homo sapiens (human) [GN:hsa]
Gene
39  ACAT2; acetyl-CoA acetyltransferase 2 [KO:K00626] [EC:2.3.1.9]
38  ACAT1; acetyl-CoA acetyltransferase 1 [KO:K00626] [EC:2.3.1.9]
3157  HMGCS1; 3-hydroxy-3-methylglutaryl-CoA synthase 1 [KO:K01641] [EC:2.3.3.10]
3158  HMGCS2; 3-hydroxy-3-methylglutaryl-CoA synthase 2 [KO:K01641] [EC:2.3.3.10]
3156  HMGCR; 3-hydroxy-3-methylglutaryl-CoA reductase [KO:K00021] [EC:1.1.1.34]
4598  MVK; mevalonate kinase [KO:K00869] [EC:2.7.1.36]
10654  PMVK; phosphomevalonate kinase [KO:K13273] [EC:2.7.4.2]
4597  MVD; mevalonate diphosphate decarboxylase [KO:K01597] [EC:4.1.1.33]
3422  IDI1; isopentenyl-diphosphate delta isomerase 1 [KO:K01823] [EC:5.3.3.2]
91734  IDI2; isopentenyl-diphosphate delta isomerase 2 [KO:K01823] [EC:5.3.3.2]
2224  FDPS; farnesyl diphosphate synthase [KO:K00787] [EC:2.5.1.10 2.5.1.1]
9453  GGPS1; geranylgeranyl diphosphate synthase 1 [KO:K00804] [EC:2.5.1.29 2.5.1.10 2.5.1.1]
23590  PDSS1; decaprenyl diphosphate synthase subunit 1 [KO:K12504] [EC:2.5.1.91]
57107  PDSS2; decaprenyl diphosphate synthase subunit 2 [KO:K12505] [EC:2.5.1.91]
79947  DHDDS; dehydrodolichyl diphosphate synthase subunit [KO:K11778] [EC:2.5.1.87]
116150  NUS1; NUS1, dehydrodolichyl diphosphate synthase subunit [KO:K19177] [EC:2.5.1.87]
2339  FNTA; farnesyltransferase, CAAX box, alpha [KO:K05955] [EC:2.5.1.59 2.5.1.58]
2342  FNTB; farnesyltransferase, CAAX box, beta [KO:K05954] [EC:2.5.1.58]
9986  RCE1; Ras converting CAAX endopeptidase 1 [KO:K08658] [EC:3.4.22.-]
10269  ZMPSTE24; zinc metallopeptidase STE24 [KO:K06013] [EC:3.4.24.84]
23463  ICMT; isoprenylcysteine carboxyl methyltransferase [KO:K00587] [EC:2.1.1.100]
51449  PCYOX1; prenylcysteine oxidase 1 [KO:K05906] [EC:1.8.3.6 1.8.3.5]
Compound
C00022  Pyruvate
C00024  Acetyl-CoA
C00118  D-Glyceraldehyde 3-phosphate
C00129  Isopentenyl diphosphate
C00235  Dimethylallyl diphosphate
C00332  Acetoacetyl-CoA
C00341  Geranyl diphosphate
C00353  Geranylgeranyl diphosphate
C00356  (S)-3-Hydroxy-3-methylglutaryl-CoA
C00418  (R)-Mevalonate
C00448  trans,trans-Farnesyl diphosphate
C00621  Dolichyl diphosphate
C01107  (R)-5-Phosphomevalonate
C01126  (2E,6E)-Farnesol
C01143  (R)-5-Diphosphomevalonate
C01230  all-trans-Hexaprenyl diphosphate
C02321  cis-1,4-Polyisoprene
C03461  2-trans,6-trans-Farnesal
C04145  all-trans-Nonaprenyl diphosphate
C04146  all-trans-Octaprenyl diphosphate
C04216  all-trans-Heptaprenyl diphosphate
C04506  Protein C-terminal S-farnesyl-L-cysteine
C04574  di-trans,poly-cis-Undecaprenyl diphosphate
C04748  Protein C-terminal S-farnesyl-L-cysteine methyl ester
C05427  Phytyl diphosphate
C05859  Dehydrodolichol diphosphate
C11434  2-C-Methyl-D-erythritol 4-phosphate
C11435  4-(Cytidine 5'-diphospho)-2-C-methyl-D-erythritol
C11436  2-Phospho-4-(cytidine 5'-diphospho)-2-C-methyl-D-erythritol
C11437  1-Deoxy-D-xylulose 5-phosphate
C11453  2-C-Methyl-D-erythritol 2,4-cyclodiphosphate
C11811  1-Hydroxy-2-methyl-2-butenyl 4-diphosphate
C16521  Isoprene
C16826  2-cis,6-trans-Farnesyl diphosphate
C17432  all-trans-Decaprenyl diphosphate
C18321  (2Z,6Z,10Z,14Z,18Z,22Z,26Z,30Z,34E)-Decaprenyl diphosphate
C19691  Farnesylcysteine
C19760  (2Z,6Z)-Farnesyl diphosphate
C19852  tritrans,heptacis-Undecaprenyl diphosphate
C19853  trans,polycis-Polyprenyl diphosphate
C20120  S-Farnesyl protein
C20121  trans,trans-Farnesyl phosphate
C20345  Isopentenyl phosphate
C20847  (R)-Mevalonate 3-phosphate
C20848  (R)-Mevalonate 3,5-bisphosphate
Reference
  Authors
Lange BM, Ghassemian M.
  Title
Genome organization in Arabidopsis thaliana: a survey for genes involved in isoprenoid and chlorophyll metabolism.
  Journal
Plant Mol Biol 51:925-48 (2003)
DOI:10.1023/A:1023005504702
Reference
  Authors
Saiki R, Nagata A, Kainou T, Matsuda H, Kawamukai M
  Title
Characterization of solanesyl and decaprenyl diphosphate synthases in mice and humans.
  Journal
FEBS J 272:5606-22 (2005)
DOI:10.1111/j.1742-4658.2005.04956.x
Reference
PMID:9858571
  Authors
Sato M, Sato K, Nishikawa S, Hirata A, Kato J, Nakano A
  Title
The yeast RER2 gene, identified by endoplasmic reticulum protein localization mutations, encodes cis-prenyltransferase, a key enzyme in dolichol synthesis.
  Journal
Mol Cell Biol 19:471-83 (1999)
DOI:10.1128/MCB.19.1.471
Reference
  Authors
Vannice JC, Skaff DA, Keightley A, Addo JK, Wyckoff GJ, Miziorko HM
  Title
Identification in Haloferax volcanii of phosphomevalonate decarboxylase and isopentenyl phosphate kinase as catalysts of the terminal enzyme reactions in an  archaeal alternate mevalonate pathway.
  Journal
J Bacteriol 196:1055-63 (2014)
DOI:10.1128/JB.01230-13
KO pathway
ko00900   

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