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
Terpenoid backbone biosynthesis
hsa00900

All organismsOrtholog table
Module
C5 isoprenoid biosynthesis, mevalonate pathway [PATH:hsa00900]
C10-C20 isoprenoid biosynthesis, non-plant eukaryotes [PATH:hsa00900]
Disease
H00206  
Mevalonate kinase deficiency
H00663  
Restrictive dermopathy
H00999  
Coenzyme Q10 deficiency
H01123  
HMG-CoA synthase (HMGCS) deficiency
Drug
D00359  
Lovastatin (USP/INN)
D00434  
Simvastatin (JP16/USP/INN)
D00887  
Atorvastatin calcium (USAN)
D00889  
Cerivastatin sodium (JAN/USAN)
D00892  
Fluvastatin sodium (JAN/USAN)
D00893  
Pravastatin sodium (JP16/USAN)
D00939  
Alendronate sodium hydrate (JP16)
D00941  
Pamidronate disodium hydrate (JAN)
D00942  
Risedronate sodium (USP)
D01862  
Pitavastatin calcium (JAN)
D01915  
Rosuvastatin calcium (JAN/USAN)
D01968  
Zoledronic acid hydrate (JAN)
D02258  
Atorvastatin calcium hydrate (JP16)
D03234  
Sodium risedronate hydrate (JP16)
D03601  
Crilvastatin (USAN/INN)
D03643  
Dalvastatin (USAN/INN)
D03720  
Tipifarnib (USAN/INN)
D03816  
Tenivastatin calcium (USAN)
D04486  
Ibandronate sodium hydrate (JAN)
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)
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)
Other DBs
BSID: 
GO: 
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 (soluble) [KO:K01641] [EC:2.3.3.10]
3158  
HMGCS2; 3-hydroxy-3-methylglutaryl-CoA synthase 2 (mitochondrial) [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 (diphospho) 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; prenyl (decaprenyl) diphosphate synthase, subunit 1 [KO:K12504] [EC:2.5.1.91]
57107  
PDSS2; prenyl (decaprenyl) diphosphate synthase, subunit 2 [KO:K12505] [EC:2.5.1.91]
79947  
DHDDS; dehydrodolichyl diphosphate synthase [KO:K11778] [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  
Rubber particle
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)
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)
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)
Reference
  Authors
Vannice JC, Skaff DA, Keightley A, Addo J, Wyckoff GJ, Miziorko HM
  Title
Identification in Haloferax volcanii of Phosphomevalonate Decarboxylase and Isopentenyl Phosphate Kinase as Catalysts of the Terminal Enzymatic Reactions in  an Archaeal Alternate Mevalonate Pathway.
  Journal
J Bacteriol (2013)
KO pathway
 

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