KEGG   PATHWAY: hsa00270Help
Entry
hsa00270                    Pathway                                

Name
Cysteine and methionine metabolism - Homo sapiens (human)
Description
Cysteine and methionine are sulfur-containing amino acids. Cysteine is synthesized from serine through different pathways in different organism groups. In bacteria and plants, cysteine is converted from serine (via acetylserine) by transfer of hydrogen sulfide [MD:M00021]. In animals, methionine-derived homocysteine is used as sulfur source and its condensation product with serine (cystathionine) is converted to cysteine [MD:M00338]. Cysteine is metabolized to pyruvate in multiple routes. Methionine is an essential amino acid, which animals cannot synthesize. In bacteria and plants, methionine is synthesized from aspartate [MD:M00017]. S-Adenosylmethionine (SAM), synthesized from methionine and ATP, is a methyl group donor in many important transfer reactions including DNA methylation for regulation of gene expression. SAM may also be used to regenerate methionine in the methionine salvage pathway [MD:M00034].
Class
Metabolism; Amino acid metabolism
BRITE hierarchy
Pathway map
hsa00270  Cysteine and methionine metabolism
hsa00270

Ortholog table
Module
hsa_M00034  Methionine salvage pathway [PATH:hsa00270]
hsa_M00035  Methionine degradation [PATH:hsa00270]
hsa_M00338  Cysteine biosynthesis, homocysteine + serine => cysteine [PATH:hsa00270]
Disease
H00147  Sialuria
H00182  Cystathioninuria
H00183  Homocystinuria
H00184  Hypermethioninemia
H00597  Snyder-Robinson syndrome
H01285  Methylcobalamin deficiency type G
Drug
D03021  Azacitidine (JAN/USAN/INN)
D03665  Decitabine (USAN/INN)
D07258  Mitoguazone (INN)
Other DBs
BSID: 104488
GO: 0006534 0006555
Organism
Homo sapiens (human) [GN:hsa]
Gene
1491  CTH; cystathionine gamma-lyase [KO:K01758] [EC:4.4.1.1]
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]
635  BHMT; betaine--homocysteine S-methyltransferase [KO:K00544] [EC:2.1.1.5]
23743  BHMT2; betaine--homocysteine S-methyltransferase 2 [KO:K00547] [EC:2.1.1.10]
4548  MTR; 5-methyltetrahydrofolate-homocysteine methyltransferase [KO:K00548] [EC:2.1.1.13]
27430  MAT2B; methionine adenosyltransferase 2B [KO:K00789] [EC:2.5.1.6]
4143  MAT1A; methionine adenosyltransferase 1A [KO:K00789] [EC:2.5.1.6]
4144  MAT2A; methionine adenosyltransferase 2A [KO:K00789] [EC:2.5.1.6]
262  AMD1; adenosylmethionine decarboxylase 1 [KO:K01611] [EC:4.1.1.50]
6723  SRM; spermidine synthase [KO:K00797] [EC:2.5.1.16]
6611  SMS; spermine synthase [KO:K00802] [EC:2.5.1.22]
4507  MTAP; methylthioadenosine phosphorylase [KO:K00772] [EC:2.4.2.28]
84245  MRI1; methylthioribose-1-phosphate isomerase 1 [KO:K08963] [EC:5.3.1.23]
51074  APIP; APAF1 interacting protein [KO:K08964] [EC:4.2.1.109]
58478  ENOPH1; enolase-phosphatase 1 [KO:K09880] [EC:3.1.3.77]
55256  ADI1; acireductone dioxygenase 1 [KO:K08967] [EC:1.13.11.54 1.13.11.53]
6898  TAT; tyrosine aminotransferase [KO:K00815] [EC:2.6.1.5]
259307  IL4I1; interleukin 4 induced 1 [KO:K03334] [EC:1.4.3.2]
1786  DNMT1; DNA methyltransferase 1 [KO:K00558] [EC:2.1.1.37]
1788  DNMT3A; DNA methyltransferase 3 alpha [KO:K17398] [EC:2.1.1.37]
1789  DNMT3B; DNA methyltransferase 3 beta [KO:K17399] [EC:2.1.1.37]
23382  AHCYL2; adenosylhomocysteinase like 2 [KO:K01251] [EC:3.3.1.1]
10768  AHCYL1; adenosylhomocysteinase like 1 [KO:K01251] [EC:3.3.1.1]
191  AHCY; adenosylhomocysteinase [KO:K01251] [EC:3.3.1.1]
587  BCAT2; branched chain amino acid transaminase 2 [KO:K00826] [EC:2.6.1.42]
586  BCAT1; branched chain amino acid transaminase 1 [KO:K00826] [EC:2.6.1.42]
64902  AGXT2; alanine--glyoxylate aminotransferase 2 [KO:K00827] [EC:2.6.1.40 2.6.1.44]
2729  GCLC; glutamate-cysteine ligase catalytic subunit [KO:K11204] [EC:6.3.2.2]
2730  GCLM; glutamate-cysteine ligase modifier subunit [KO:K11205]
2937  GSS; glutathione synthetase [KO:K21456] [EC:6.3.2.3]
1036  CDO1; cysteine dioxygenase type 1 [KO:K00456] [EC:1.13.11.20]
2805  GOT1; glutamic-oxaloacetic transaminase 1 [KO:K14454] [EC:2.6.1.1]
2806  GOT2; glutamic-oxaloacetic transaminase 2 [KO:K14455] [EC:2.6.1.1]
4357  MPST; mercaptopyruvate sulfurtransferase [KO:K01011] [EC:2.8.1.2 2.8.1.1]
7263  TST; thiosulfate sulfurtransferase [KO:K01011] [EC:2.8.1.2 2.8.1.1]
160287  LDHAL6A; lactate dehydrogenase A like 6A [KO:K00016] [EC:1.1.1.27]
92483  LDHAL6B; lactate dehydrogenase A like 6B [KO:K00016] [EC:1.1.1.27]
3939  LDHA; lactate dehydrogenase A [KO:K00016] [EC:1.1.1.27]
3945  LDHB; lactate dehydrogenase B [KO:K00016] [EC:1.1.1.27]
3948  LDHC; lactate dehydrogenase C [KO:K00016] [EC:1.1.1.27]
4190  MDH1; malate dehydrogenase 1 [KO:K00025] [EC:1.1.1.37]
4191  MDH2; malate dehydrogenase 2 [KO:K00026] [EC:1.1.1.37]
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]
Compound
C00019  S-Adenosyl-L-methionine
C00021  S-Adenosyl-L-homocysteine
C00022  Pyruvate
C00041  L-Alanine
C00049  L-Aspartate
C00051  Glutathione
C00059  Sulfate
C00065  L-Serine
C00073  L-Methionine
C00094  Sulfite
C00097  L-Cysteine
C00109  2-Oxobutanoate
C00155  L-Homocysteine
C00170  5'-Methylthioadenosine
C00263  L-Homoserine
C00283  Hydrogen sulfide
C00409  Methanethiol
C00441  L-Aspartate 4-semialdehyde
C00491  L-Cystine
C00506  L-Cysteate
C00606  3-Sulfino-L-alanine
C00793  D-Cysteine
C00957  Mercaptopyruvate
C00979  O-Acetyl-L-serine
C01005  O-Phospho-L-serine
C01077  O-Acetyl-L-homoserine
C01118  O-Succinyl-L-homoserine
C01137  S-Adenosylmethioninamine
C01180  4-Methylthio-2-oxobutanoic acid
C01234  1-Aminocyclopropane-1-carboxylate
C01817  L-Homocystine
C01962  Thiocysteine
C02218  Dehydroalanine
C02291  L-Cystathionine
C02356  (S)-2-Aminobutanoate
C02989  L-Methionine S-oxide
C03082  4-Phospho-L-aspartate
C03089  5-Methylthio-D-ribose
C03145  N-Formylmethionine
C03539  S-Ribosyl-L-homocysteine
C04188  S-Methyl-5-thio-D-ribose 1-phosphate
C04582  S-Methyl-5-thio-D-ribulose 1-phosphate
C05524  Aminoacyl-L-methionine
C05526  S-Glutathionyl-L-cysteine
C05527  3-Sulfinylpyruvate
C05528  3-Sulfopyruvate
C05823  3-Mercaptolactate
C05824  S-Sulfo-L-cysteine
C06547  Ethylene
C08276  3-(Methylthio)propanoate
C09306  Sulfur dioxide
C11481  HSO3-
C11499  (S)-3-Sulfolactate
C11537  (2R)-3-Sulfolactate
C15606  1,2-Dihydroxy-5-(methylthio)pent-1-en-3-one
C15650  2,3-Diketo-5-methylthiopentyl-1-phosphate
C15651  2-Hydroxy-3-keto-5-methylthiopentenyl-1-phosphate
C18049  N-Acyl-L-homoserine lactone
C19787  5'-S-Methyl-5'-thioinosine
C21015  gamma-L-Glutamyl-L-2-aminobutyrate
C21016  Ophthalmate
Reference
  Authors
Sekowska A, Denervaud V, Ashida H, Michoud K, Haas D, Yokota A, Danchin A
  Title
Bacterial variations on the methionine salvage pathway.
  Journal
BMC Microbiol 4:9 (2004)
DOI:10.1186/1471-2180-4-9
Reference
  Authors
Ashida H, Saito Y, Kojima C, Kobayashi K, Ogasawara N, Yokota A.
  Title
A functional link between RuBisCO-like protein of Bacillus and photosynthetic RuBisCO.
  Journal
Science 302:286-90 (2003)
DOI:10.1126/science.1086997
Reference
  Authors
Sekowska A, Danchin A.
  Title
The methionine salvage pathway in Bacillus subtilis.
  Journal
BMC Microbiol 2:8 (2002)
DOI:10.1186/1471-2180-2-8
Reference
  Authors
Berger BJ, English S, Chan G, Knodel MH.
  Title
Methionine regeneration and aminotransferases in Bacillus subtilis, Bacillus cereus, and Bacillus anthracis.
  Journal
J Bacteriol 185:2418-31 (2003)
DOI:10.1128/JB.185.8.2418-2431.2003
Reference
  Authors
Goyer A, Collakova E, Shachar-Hill Y, Hanson AD
  Title
Functional characterization of a methionine gamma-lyase in Arabidopsis and its implication in an alternative to the reverse trans-sulfuration pathway.
  Journal
Plant Cell Physiol 48:232-42 (2007)
DOI:10.1093/pcp/pcl055
Reference
  Authors
Rebeille F, Jabrin S, Bligny R, Loizeau K, Gambonnet B, Van Wilder V, Douce R, Ravanel S
  Title
Methionine catabolism in Arabidopsis cells is initiated by a gamma-cleavage process and leads to S-methylcysteine and isoleucine syntheses.
  Journal
Proc Natl Acad Sci U S A 103:15687-92 (2006)
DOI:10.1073/pnas.0606195103
Reference
  Authors
Pirkov I, Norbeck J, Gustafsson L, Albers E
  Title
A complete inventory of all enzymes in the eukaryotic methionine salvage pathway.
  Journal
FEBS J 275:4111-20 (2008)
DOI:10.1111/j.1742-4658.2008.06552.x
Reference
  Authors
Ashida H, Saito Y, Kojima C, Yokota A
  Title
Enzymatic characterization of 5-methylthioribulose-1-phosphate dehydratase of the methionine salvage pathway in Bacillus subtilis.
  Journal
Biosci Biotechnol Biochem 72:959-67 (2008)
DOI:10.1271/bbb.70651
Reference
  Authors
Kitabatake M, So MW, Tumbula DL, Soll D
  Title
Cysteine biosynthesis pathway in the archaeon Methanosarcina barkeri encoded by acquired bacterial genes?
  Journal
J Bacteriol 182:143-5 (2000)
DOI:10.1128/JB.182.1.143-145.2000
Reference
  Authors
Mino K, Ishikawa K
  Title
Characterization of a novel thermostable O-acetylserine sulfhydrylase from Aeropyrum pernix K1.
  Journal
J Bacteriol 185:2277-84 (2003)
DOI:10.1128/JB.185.7.2277-2284.2003
Reference
  Authors
Tanabe S
  Title
Development of assay methods for endogenous inorganic sulfur compounds and sulfurtransferases and evaluation of the physiological functions of bound sulfur.
  Journal
Yakugaku Zasshi 128:881-900 (2008)
DOI:10.1248/yakushi.128.881
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
Gutierrez JA, Crowder T, Rinaldo-Matthis A, Ho MC, Almo SC, Schramm VL
  Title
Transition state analogs of 5'-methylthioadenosine nucleosidase disrupt quorum sensing.
  Journal
Nat Chem Biol 5:251-7 (2009)
DOI:10.1038/nchembio.153
KO pathway
ko00270   

DBGET integrated database retrieval system