KEGG   PATHWAY: hsa00260Help
Entry
hsa00260                    Pathway                                

Name
Glycine, serine and threonine metabolism - Homo sapiens (human)
Description
Serine is derived from 3-phospho-D-glycerate, an intermediate of glycolysis [MD:M00020], and glycine is derived from serine. Threonine is an essential amino acid, which animals cannot synthesize. In bacteria and plants, threonine is derived from aspartate [MD:M00018].
Class
Metabolism; Amino acid metabolism
BRITE hierarchy
Pathway map
hsa00260  Glycine, serine and threonine metabolism
hsa00260

Ortholog table
Module
hsa_M00020  Serine biosynthesis, glycerate-3P => serine [PATH:hsa00260]
Disease
H00117  Primary hyperoxaluria (HP)
H00182  Cystathioninuria
H00183  Homocystinuria
H00184  Hypermethioninemia
H00191  Nonketotic hyperglycinemia
H00834  Guanidinoacetate methyltransferase (GAMT) deficiency
H00849  Creatine deficiency syndrome
H01003  Dimethylglycine dehydrogenase deficiency (DMGDHD)
H01079  3-Phosphoglycerate dehydrogenase (3-PGDH) deficiency
H01082  Phosphoserine aminotransferase (PSAT) deficiency
H01951  Glycogen storage disease type X
Drug
D00505  Phenelzine sulfate (USP)
D00826  Tranylcypromine sulfate (USP XXI)
D01888  Safrazine hydrochloride (JAN)
D02559  Toloxatone (INN)
D02560  Brofaromine (INN)
D02561  Moclobemide (USAN/INN)
D02563  Befloxatone (INN)
D02579  Iproniazid (INN)
D02580  Isocarboxazid (INN)
D02581  Cimoxatone (INN)
D03239  Ladostigil tartrate (USAN)
D03248  Clorgiline (INN)
D03409  Caroxazone (USAN/INN)
D05033  Milacemide hydrochloride (USAN)
D07337  Nialamide (INN)
D07338  Iproclozide (INN)
D08085  Iproniazid phosphate
D08349  Phenelzine (BAN)
D08392  Pirlindole (INN)
D08393  Pirlindole hydrochloride
D08625  Tranylcypromine (INN)
Other DBs
BSID: 82949
GO: 0006544 0006563 0070178 0006566
Organism
Homo sapiens (human) [GN:hsa]
Gene
6472  SHMT2; serine hydroxymethyltransferase 2 [KO:K00600] [EC:2.1.2.1]
6470  SHMT1; serine hydroxymethyltransferase 1 [KO:K00600] [EC:2.1.2.1]
189  AGXT; alanine-glyoxylate aminotransferase [KO:K00830] [EC:2.6.1.51 2.6.1.45 2.6.1.44]
9380  GRHPR; glyoxylate and hydroxypyruvate reductase [KO:K00049] [EC:1.1.1.81 1.1.1.79]
132158  GLYCTK; glycerate kinase [KO:K11529] [EC:2.7.1.165]
5223  PGAM1; phosphoglycerate mutase 1 [KO:K01834] [EC:5.4.2.11]
5224  PGAM2; phosphoglycerate mutase 2 [KO:K01834] [EC:5.4.2.11]
441531  PGAM4; phosphoglycerate mutase family member 4 [KO:K01834] [EC:5.4.2.11]
669  BPGM; bisphosphoglycerate mutase [KO:K01837] [EC:5.4.2.11 5.4.2.4]
26227  PHGDH; phosphoglycerate dehydrogenase [KO:K00058] [EC:1.1.1.399 1.1.1.95]
29968  PSAT1; phosphoserine aminotransferase 1 [KO:K00831] [EC:2.6.1.52]
5723  PSPH; phosphoserine phosphatase [KO:K01079] [EC:3.1.3.3]
23464  GCAT; glycine C-acetyltransferase [KO:K00639] [EC:2.3.1.29]
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]
4129  MAOB; monoamine oxidase B [KO:K00274] [EC:1.4.3.4]
4128  MAOA; monoamine oxidase A [KO:K00274] [EC:1.4.3.4]
8639  AOC3; amine oxidase, copper containing 3 [KO:K00276] [EC:1.4.3.21]
314  AOC2; amine oxidase, copper containing 2 [KO:K00276] [EC:1.4.3.21]
2731  GLDC; glycine decarboxylase [KO:K00281] [EC:1.4.4.2]
275  AMT; aminomethyltransferase [KO:K00605] [EC:2.1.2.10]
1738  DLD; dihydrolipoamide dehydrogenase [KO:K00382] [EC:1.8.1.4]
2653  GCSH; glycine cleavage system protein H [KO:K02437]
1610  DAO; D-amino acid oxidase [KO:K00273] [EC:1.4.3.3]
64902  AGXT2; alanine--glyoxylate aminotransferase 2 [KO:K00827] [EC:2.6.1.40 2.6.1.44]
2628  GATM; glycine amidinotransferase [KO:K00613] [EC:2.1.4.1]
2593  GAMT; guanidinoacetate N-methyltransferase [KO:K00542] [EC:2.1.1.2]
55349  CHDH; choline dehydrogenase [KO:K00108] [EC:1.1.99.1]
501  ALDH7A1; aldehyde dehydrogenase 7 family member A1 [KO:K14085] [EC:1.2.1.3 1.2.1.8 1.2.1.31]
635  BHMT; betaine--homocysteine S-methyltransferase [KO:K00544] [EC:2.1.1.5]
29958  DMGDH; dimethylglycine dehydrogenase [KO:K00315] [EC:1.5.8.4]
51268  PIPOX; pipecolic acid and sarcosine oxidase [KO:K00306] [EC:1.5.3.7 1.5.3.1]
1757  SARDH; sarcosine dehydrogenase [KO:K00314] [EC:1.5.8.3]
27232  GNMT; glycine N-methyltransferase [KO:K00552] [EC:2.1.1.20]
875  CBS; cystathionine-beta-synthase [KO:K01697] [EC:4.2.1.22]
102724560  CBSL; cystathionine-beta-synthase like [KO:K01697] [EC:4.2.1.22]
1491  CTH; cystathionine gamma-lyase [KO:K01758] [EC:4.4.1.1]
10993  SDS; serine dehydratase [KO:K17989] [EC:4.3.1.19 4.3.1.17]
113675  SDSL; serine dehydratase like [KO:K17989] [EC:4.3.1.19 4.3.1.17]
63826  SRR; serine racemase [KO:K12235] [EC:5.1.1.18]
Compound
C00011  CO2
C00014  Ammonia
C00022  Pyruvate
C00037  Glycine
C00048  Glyoxylate
C00049  L-Aspartate
C00065  L-Serine
C00078  L-Tryptophan
C00097  L-Cysteine
C00101  Tetrahydrofolate
C00109  2-Oxobutanoate
C00114  Choline
C00143  5,10-Methylenetetrahydrofolate
C00168  Hydroxypyruvate
C00188  L-Threonine
C00197  3-Phospho-D-glycerate
C00213  Sarcosine
C00258  D-Glycerate
C00263  L-Homoserine
C00300  Creatine
C00430  5-Aminolevulinate
C00441  L-Aspartate 4-semialdehyde
C00546  Methylglyoxal
C00576  Betaine aldehyde
C00581  Guanidinoacetate
C00631  2-Phospho-D-glycerate
C00719  Betaine
C00740  D-Serine
C00986  1,3-Diaminopropane
C01005  O-Phospho-L-serine
C01026  N,N-Dimethylglycine
C01102  O-Phospho-L-homoserine
C01242  S-Aminomethyldihydrolipoylprotein
C01726  D-Lombricine
C01888  Aminoacetone
C02051  Lipoylprotein
C02291  L-Cystathionine
C02737  Phosphatidylserine
C02855  N-Phospho-D-lombricine
C02972  Dihydrolipoylprotein
C03082  4-Phospho-L-aspartate
C03194  (R)-1-Aminopropan-2-ol
C03232  3-Phosphonooxypyruvate
C03283  L-2,4-Diaminobutanoate
C03508  L-2-Amino-3-oxobutanoic acid
C05519  L-Allothreonine
C06231  Ectoine
C06442  N(gamma)-Acetyldiaminobutyrate
C16432  5-Hydroxyectoine
C19929  N(alpha)-Acetyl-L-2,4-diaminobutyrate
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
Kuhlmann AU, Bremer E.
  Title
Osmotically regulated synthesis of the compatible solute ectoine in Bacillus pasteurii and related Bacillus spp.
  Journal
Appl Environ Microbiol 68:772-83 (2002)
DOI:10.1128/AEM.68.2.772-783.2002
Reference
  Authors
Canovas D, Borges N, Vargas C, Ventosa A, Nieto JJ, Santos H.
  Title
Role of Ngamma-acetyldiaminobutyrate as an enzyme stabilizer and an intermediate in the biosynthesis of hydroxyectoine.
  Journal
Appl Environ Microbiol 65:3774-9 (1999)
Reference
  Authors
Bursy J, Pierik AJ, Pica N, Bremer E.
  Title
Osmotically induced synthesis of the compatible solute hydroxyectoine is mediated by an evolutionarily conserved ectoine hydroxylase.
  Journal
J Biol Chem 282:31147-55 (2007)
DOI:10.1074/jbc.M704023200
Reference
  Authors
Garcia-Estepa R, Argandona M, Reina-Bueno M, Capote N, Iglesias-Guerra F, Nieto JJ, Vargas C.
  Title
The ectD gene, which is involved in the synthesis of the compatible solute hydroxyectoine, is essential for thermoprotection of the halophilic bacterium Chromohalobacter salexigens.
  Journal
J Bacteriol 188:3774-84 (2006)
DOI:10.1128/JB.00136-06
Reference
  Authors
Arevalo-Rodriguez M, Pan X, Boeke JD, Heitman J
  Title
FKBP12 controls aspartate pathway flux in Saccharomyces cerevisiae to prevent toxic intermediate accumulation.
  Journal
Eukaryot Cell 3:1287-96 (2004)
DOI:10.1128/EC.3.5.1287-1296.2004
Reference
PMID:7639721
  Authors
Rees WD, Hay SM
  Title
The biosynthesis of threonine by mammalian cells: expression of a complete bacterial biosynthetic pathway in an animal cell.
  Journal
Biochem J 309 ( Pt 3):999-1007 (1995)
DOI:10.1042/bj3090999
Reference
  Authors
Jacques SL, Nieman C, Bareich D, Broadhead G, Kinach R, Honek JF, Wright GD
  Title
Characterization of yeast homoserine dehydrogenase, an antifungal target: the invariant histidine 309 is important for enzyme integrity.
  Journal
Biochim Biophys Acta 1544:28-41 (2001)
DOI:10.1016/S0167-4838(00)00203-X
Reference
  Authors
Chen X, Jhee KH, Kruger WD
  Title
Production of the neuromodulator H2S by cystathionine beta-synthase via the condensation of cysteine and homocysteine.
  Journal
J Biol Chem 279:52082-6 (2004)
DOI:10.1074/jbc.C400481200
KO pathway
ko00260   

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