MiMeDB: Showing metabocard for L-Cystathionine (MMDBc0000495)

Record Information

Version

1.0

Status

Detected and Quantified

Creation Date

2020-12-10 18:39:04 UTC

Update Date

2024-04-30 19:32:37 UTC

Metabolite ID

MMDBc0000495

Metabolite Identification

Common Name

L-Cystathionine

Description

Cystathionine is a dipeptide formed by serine and homocysteine. Cystathioninuria is a prominent manifestation of vitamin-B6 deficiency. The transsulfuration of methionine yields homocysteine, which combines with serine to form cystathionine, the proximate precursor of cysteine through the enzymatic activity of cystathionase. In conditions in which cystathionine gamma-synthase or cystathionase is deficient, for example, there is cystathioninuria. Although cystathionine has not been detected in normal human serum or plasma by most conventional methods, gas chromatographic/mass spectrometric methodology detected a mean concentration of cystathionine in normal human serum of 140 nM, with a range of 65 to 301 nM. Cystathionine concentrations in CSF have been 10, 1, and 0.5 uM, and "not detected". Only traces (i.e., <1 uM) of cystathionine are present in normal CSF.587. Gamma-cystathionase deficiency (also known as Cystathioninuria), which is an autosomal recessive disorder (NIH: 2428), provided the first instance in which, in a human, the major biochemical abnormality due to a defined enzyme defect was clearly shown to be alleviated by administration of large doses of pyridoxine. The response in gamma-cystathionase-deficient patients is not attributable to correction of a preexisting deficiency of this vitamin (OMMBID, Chap. 88).

Structure

MOL 3D MOL SDF 3D SDF PDB 3D PDB SMILES InChI

 
  Mrv1652309032023522D          
 
 14 13  0  0  1  0            999 V2000
   11.4612   -9.9135    0.0000 C   0  0  1  0  0  0  0  0  0  0  0  0
   12.1305   -9.4952    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   10.7501   -9.4952    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   11.4612  -10.7501    0.0000 N   0  0  0  0  0  0  0  0  0  0  0  0
   12.8416   -9.9135    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   10.0390   -9.9135    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   10.7501   -8.6586    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   13.5945   -9.4952    0.0000 S   0  0  0  0  0  0  0  0  0  0  0  0
   14.3056   -9.9135    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   15.0167   -9.4952    0.0000 C   0  0  1  0  0  0  0  0  0  0  0  0
   15.7278   -9.9135    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   15.0167   -8.5750    0.0000 N   0  0  0  0  0  0  0  0  0  0  0  0
   16.4389   -9.4952    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   15.7278  -10.7501    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0  0  0  0
  1  3  1  0  0  0  0
  1  4  1  6  0  0  0
  2  5  1  0  0  0  0
  3  6  1  0  0  0  0
  3  7  2  0  0  0  0
  5  8  1  0  0  0  0
  8  9  1  0  0  0  0
  9 10  1  0  0  0  0
 10 11  1  0  0  0  0
 10 12  1  6  0  0  0
 11 13  1  0  0  0  0
 11 14  2  0  0  0  0
M  END
> <DATABASE_ID>
MMDBc0000495

> <DATABASE_NAME>
MIME

> <SMILES>
N[C@@H](CCSC[C@H](N)C(O)=O)C(O)=O

> <INCHI_IDENTIFIER>
InChI=1S/C7H14N2O4S/c8-4(6(10)11)1-2-14-3-5(9)7(12)13/h4-5H,1-3,8-9H2,(H,10,11)(H,12,13)/t4-,5-/m0/s1

> <INCHI_KEY>
ILRYLPWNYFXEMH-WHFBIAKZSA-N

> <FORMULA>
C7H14N2O4S

> <MOLECULAR_WEIGHT>
222.262

> <EXACT_MASS>
222.067427636

> <JCHEM_ACCEPTOR_COUNT>
6

> <JCHEM_ATOM_COUNT>
28

> <JCHEM_AVERAGE_POLARIZABILITY>
22.01922512395099

> <JCHEM_BIOAVAILABILITY>
1

> <JCHEM_DONOR_COUNT>
4

> <JCHEM_FORMAL_CHARGE>
0

> <JCHEM_GHOSE_FILTER>
0

> <JCHEM_IUPAC>
(2S)-2-amino-4-{[(2R)-2-amino-2-carboxyethyl]sulfanyl}butanoic acid

> <ALOGPS_LOGP>
-4.01

> <JCHEM_LOGP>
-5.818502121951177

> <ALOGPS_LOGS>
-1.11

> <JCHEM_MDDR_LIKE_RULE>
0

> <JCHEM_NUMBER_OF_RINGS>
0

> <JCHEM_PHYSIOLOGICAL_CHARGE>
0

> <JCHEM_PKA>
2.514696511113594

> <JCHEM_PKA_STRONGEST_ACIDIC>
1.793798721038319

> <JCHEM_PKA_STRONGEST_BASIC>
9.659588767699923

> <JCHEM_POLAR_SURFACE_AREA>
126.64000000000001

> <JCHEM_REFRACTIVITY>
51.568900000000006

> <JCHEM_ROTATABLE_BOND_COUNT>
7

> <JCHEM_RULE_OF_FIVE>
1

> <ALOGPS_SOLUBILITY>
1.73e+01 g/l

> <JCHEM_TRADITIONAL_IUPAC>
L-cystathionine

> <JCHEM_VEBER_RULE>
0

$$$$

HEADER    PROTEIN                                 03-SEP-20   NONE
TITLE     NULL                                                        
COMPND    MOLECULE:                                                   
SOURCE    NULL                                                        
KEYWDS    NULL                                                        
EXPDTA    NULL                                                        
AUTHOR    Marvin                                                      
REVDAT   1   03-SEP-20         0                                  
HETATM    1  C   UNK     0      21.394 -18.505   0.000  0.00  0.00           C+0
HETATM    2  C   UNK     0      22.644 -17.724   0.000  0.00  0.00           C+0
HETATM    3  C   UNK     0      20.067 -17.724   0.000  0.00  0.00           C+0
HETATM    4  N   UNK     0      21.394 -20.067   0.000  0.00  0.00           N+0
HETATM    5  C   UNK     0      23.971 -18.505   0.000  0.00  0.00           C+0
HETATM    6  O   UNK     0      18.739 -18.505   0.000  0.00  0.00           O+0
HETATM    7  O   UNK     0      20.067 -16.163   0.000  0.00  0.00           O+0
HETATM    8  S   UNK     0      25.376 -17.724   0.000  0.00  0.00           S+0
HETATM    9  C   UNK     0      26.704 -18.505   0.000  0.00  0.00           C+0
HETATM   10  C   UNK     0      28.031 -17.724   0.000  0.00  0.00           C+0
HETATM   11  C   UNK     0      29.359 -18.505   0.000  0.00  0.00           C+0
HETATM   12  N   UNK     0      28.031 -16.007   0.000  0.00  0.00           N+0
HETATM   13  O   UNK     0      30.686 -17.724   0.000  0.00  0.00           O+0
HETATM   14  O   UNK     0      29.359 -20.067   0.000  0.00  0.00           O+0
CONECT    1    2    3    4                                                  
CONECT    2    1    5                                                       
CONECT    3    1    6    7                                                  
CONECT    4    1                                                            
CONECT    5    2    8                                                       
CONECT    6    3                                                            
CONECT    7    3                                                            
CONECT    8    5    9                                                       
CONECT    9    8   10                                                       
CONECT   10    9   11   12                                                  
CONECT   11   10   13   14                                                  
CONECT   12   10                                                            
CONECT   13   11                                                            
CONECT   14   11                                                            
MASTER        0    0    0    0    0    0    0    0   14    0   26    0
END

COMPND    HMDB0000099
HETATM    1  N1  UNL     1      -4.160  -0.896  -0.976  1.00  0.00           N  
HETATM    2  C1  UNL     1      -3.282  -0.248  -0.002  1.00  0.00           C  
HETATM    3  C2  UNL     1      -1.880  -0.512  -0.442  1.00  0.00           C  
HETATM    4  C3  UNL     1      -0.826   0.087   0.459  1.00  0.00           C  
HETATM    5  S1  UNL     1       0.825  -0.342  -0.217  1.00  0.00           S  
HETATM    6  C4  UNL     1       2.067   0.376   0.865  1.00  0.00           C  
HETATM    7  C5  UNL     1       3.474   0.081   0.397  1.00  0.00           C  
HETATM    8  N2  UNL     1       3.692   0.616  -0.930  1.00  0.00           N  
HETATM    9  C6  UNL     1       3.793  -1.350   0.434  1.00  0.00           C  
HETATM   10  O1  UNL     1       3.692  -2.062   1.465  1.00  0.00           O  
HETATM   11  O2  UNL     1       4.232  -1.968  -0.726  1.00  0.00           O  
HETATM   12  C7  UNL     1      -3.623   1.191   0.031  1.00  0.00           C  
HETATM   13  O3  UNL     1      -4.496   1.662  -0.757  1.00  0.00           O  
HETATM   14  O4  UNL     1      -3.002   2.056   0.919  1.00  0.00           O  
HETATM   15  H1  UNL     1      -5.030  -1.253  -0.521  1.00  0.00           H  
HETATM   16  H2  UNL     1      -3.666  -1.664  -1.479  1.00  0.00           H  
HETATM   17  H3  UNL     1      -3.458  -0.688   1.020  1.00  0.00           H  
HETATM   18  H4  UNL     1      -1.747  -0.006  -1.432  1.00  0.00           H  
HETATM   19  H5  UNL     1      -1.684  -1.594  -0.620  1.00  0.00           H  
HETATM   20  H6  UNL     1      -0.923   1.197   0.445  1.00  0.00           H  
HETATM   21  H7  UNL     1      -0.945  -0.303   1.492  1.00  0.00           H  
HETATM   22  H8  UNL     1       1.933   0.079   1.910  1.00  0.00           H  
HETATM   23  H9  UNL     1       1.963   1.497   0.847  1.00  0.00           H  
HETATM   24  H10 UNL     1       4.201   0.598   1.062  1.00  0.00           H  
HETATM   25  H11 UNL     1       3.758   1.653  -0.849  1.00  0.00           H  
HETATM   26  H12 UNL     1       2.936   0.301  -1.565  1.00  0.00           H  
HETATM   27  H13 UNL     1       4.743  -1.428  -1.421  1.00  0.00           H  
HETATM   28  H14 UNL     1      -2.586   2.920   0.592  1.00  0.00           H  
CONECT    1    2   15   16
CONECT    2    3   12   17
CONECT    3    4   18   19
CONECT    4    5   20   21
CONECT    5    6
CONECT    6    7   22   23
CONECT    7    8    9   24
CONECT    8   25   26
CONECT    9   10   10   11
CONECT   11   27
CONECT   12   13   13   14
CONECT   14   28
END

Synonyms

Value	Source
(R)-S-(2-Amino-2-carboxyethyl)-L-homocysteine	ChEBI
L-(+)-Cystathionine	ChEBI
S-(beta-Amino-beta-carboxyethyl)homocysteine	ChEBI
S-(b-Amino-b-carboxyethyl)homocysteine	Generator
S-(Β-amino-β-carboxyethyl)homocysteine	Generator
Cystathionine	HMDB
S-[(2R)-2-Amino-2-carboxyethyl]-L-homocysteine	HMDB
[R-(R,s)]-2-amino-4-[(2-amino-2-carboxyethyl)thio]-butanoate	HMDB
[R-(R,s)]-2-amino-4-[(2-amino-2-carboxyethyl)thio]-butanoic acid	HMDB

Molecular Formula

C₇H₁₄N₂O₄S

Average Mass

222.262

Monoisotopic Mass

222.067427636

IUPAC Name

(2S)-2-amino-4-{[(2R)-2-amino-2-carboxyethyl]sulfanyl}butanoic acid

Traditional Name

L-cystathionine

CAS Registry Number

Not Available

SMILES

N[C@@H](CCSC[C@H](N)C(O)=O)C(O)=O

InChI Identifier

InChI=1S/C7H14N2O4S/c8-4(6(10)11)1-2-14-3-5(9)7(12)13/h4-5H,1-3,8-9H2,(H,10,11)(H,12,13)/t4-,5-/m0/s1

InChI Key

ILRYLPWNYFXEMH-WHFBIAKZSA-N

Chemical Taxonomy

Description

Belongs to the class of organic compounds known as l-cysteine-s-conjugates. L-cysteine-S-conjugates are compounds containing L-cysteine where the thio-group is conjugated.

Kingdom

Organic compounds

Super Class

Organic acids and derivatives

Class

Carboxylic acids and derivatives

Sub Class

Amino acids, peptides, and analogues

Direct Parent

L-cysteine-S-conjugates

Alternative Parents

Substituents

L-cysteine-s-conjugate
Alpha-amino acid
L-alpha-amino acid
Thia fatty acid
Dicarboxylic acid or derivatives
Fatty acyl
Fatty acid
Amino acid
Carboxylic acid
Thioether
Dialkylthioether
Sulfenyl compound
Organic nitrogen compound
Hydrocarbon derivative
Organic oxide
Primary amine
Organosulfur compound
Organooxygen compound
Organonitrogen compound
Primary aliphatic amine
Organopnictogen compound
Organic oxygen compound
Carbonyl group
Amine
Aliphatic acyclic compound

Molecular Framework

Aliphatic acyclic compounds

External Descriptors

cystathionine (CHEBI:17482 )

Functional Ontology

Not Available

Physical Properties

State

Expected Solid

Predicted Properties

Property	Value	Source
Water Solubility	17.3 g/L	ALOGPS
logP	-4	ALOGPS
logP	-5.8	ChemAxon
logS	-1.1	ALOGPS
pKa (Strongest Acidic)	1.79	ChemAxon
pKa (Strongest Basic)	9.66	ChemAxon
Physiological Charge	0	ChemAxon
Hydrogen Acceptor Count	6	ChemAxon
Hydrogen Donor Count	4	ChemAxon
Polar Surface Area	126.64 Å²	ChemAxon
Rotatable Bond Count	7	ChemAxon
Refractivity	51.57 m³·mol⁻¹	ChemAxon
Polarizability	22.02 Å³	ChemAxon
Number of Rings	0	ChemAxon
Bioavailability	Yes	ChemAxon
Rule of Five	Yes	ChemAxon
Ghose Filter	No	ChemAxon
Veber's Rule	No	ChemAxon
MDDR-like Rule	No	ChemAxon

Spectra

Spectrum Type	Description	Splash Key	Deposition Date	View
GC-MS	GC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (4 TMS)	splash10-00fr-8950000000-640b4294e37e5a01048d	2014-06-16	View Spectrum
GC-MS	GC-MS Spectrum - GC-MS (2 TMS)	splash10-0f7c-7910000000-91f9f2b2dd44a3cdb276	2014-06-16	View Spectrum
GC-MS	GC-MS Spectrum - GC-MS (3 TMS)	splash10-00or-1940000000-c0d688e4ea87ff3e03fa	2014-06-16	View Spectrum
GC-MS	GC-MS Spectrum - GC-MS (4 TMS)	splash10-00or-0980000000-7d816de782a902900d0e	2014-06-16	View Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-00fr-8950000000-640b4294e37e5a01048d	2017-09-12	View Spectrum
GC-MS	GC-MS Spectrum - GC-MS (Non-derivatized)	splash10-0f7c-7910000000-91f9f2b2dd44a3cdb276	2017-09-12	View Spectrum
GC-MS	GC-MS Spectrum - GC-MS (Non-derivatized)	splash10-00or-1940000000-c0d688e4ea87ff3e03fa	2017-09-12	View Spectrum
GC-MS	GC-MS Spectrum - GC-MS (Non-derivatized)	splash10-00or-0980000000-7d816de782a902900d0e	2017-09-12	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive	splash10-00di-9410000000-9487e9168e86a2297931	2016-09-22	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (2 TMS) - 70eV, Positive	splash10-00dm-9665000000-608a6335880ad1c2519d	2017-10-06	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive	Not Available	2021-10-12	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_1_1) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_1_2) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_1_3) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_1_4) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_2_2) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_2_3) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_2_4) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_2_5) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_2_6) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_2_7) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_2_8) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TBDMS_1_1) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TBDMS_1_2) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TBDMS_1_3) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)	splash10-0089-1980000000-99cb7d1da067e5436b09	2012-07-24	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)	splash10-000i-9200000000-fb776970b6ef36e72725	2012-07-24	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)	splash10-000i-9200000000-fb776970b6ef36e72725	2012-07-24	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Positive	splash10-00fr-0690000000-0b572de3fcbc7e2f13cc	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Positive	splash10-000t-0900000000-5788192b32aadd177e33	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Positive	splash10-000b-4900000000-b86ac7de446a762365bd	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Positive	splash10-000j-9800000000-75ff1bc6e325692a9da9	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Positive	splash10-00kr-9100000000-6517562dc0f31a85afc8	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-IT (LC/MSD Trap XCT, Agilent Technologies) , Positive	splash10-001i-0900000000-d1628d0a880f1b6e6d90	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-IT (LC/MSD Trap XCT, Agilent Technologies) , Positive	splash10-000i-9000000000-9a2e1e54b77216e2df72	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Positive	splash10-00di-0090000000-36678c2f0bf1b01cc9d4	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) 30V, Positive	splash10-05fr-0190000000-e575f57f0d76ef6afa49	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Negative	splash10-00e9-0940000000-962ae510f06296b98878	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QTOF , negative	splash10-00e9-0940000000-27da1912eac54f485bfa	2017-09-14	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ , positive	splash10-00fr-0690000000-82fe2198c98660008c04	2017-09-14	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ , positive	splash10-000t-0900000000-58cceb69cceeff79606c	2017-09-14	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ , positive	splash10-000b-4900000000-5877787c54120dc165c9	2017-09-14	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ , positive	splash10-000j-9800000000-fd29b0fcf62b73831829	2017-09-14	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ , positive	splash10-00kr-9100000000-6517562dc0f31a85afc8	2017-09-14	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Positive	splash10-05di-1930000000-887e3ad7da04e4f1aac9	2016-09-12	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Positive	splash10-01uc-6900000000-563444ac8ab717997268	2016-09-12	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Positive	splash10-0uki-9700000000-35397d8e2ab2c6582c52	2016-09-12	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Negative	splash10-00di-0970000000-d0f0a96ca41d1f751c26	2016-09-12	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Negative	splash10-0089-3910000000-22200c974305822456d8	2016-09-12	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Negative	splash10-008l-9300000000-0dfaab5efe3438889c3c	2016-09-12	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 500 MHz, H₂O, experimental)		2012-12-04	View Spectrum
1D NMR	¹H NMR Spectrum (1D, D₂O, experimental)		2016-10-22	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, D₂O, experimental)		2016-10-22	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 100 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 100 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 1000 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 1000 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 200 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 200 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 300 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 300 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 400 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 400 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 500 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 500 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 600 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 600 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 700 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 700 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 800 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 800 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 900 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 900 MHz, D₂O, predicted)		2021-09-29	View Spectrum
2D NMR	[¹H, ¹³C]-HSQC NMR Spectrum (2D, 600 MHz, H₂O, experimental)		2012-12-04	View Spectrum

Biological Properties

Cellular Locations

Cytoplasm

Biospecimen Locations

Blood
Brain
Cerebrospinal Fluid (CSF)
Feces
Kidney
Liver
Prostate
Urine

Tissue Locations

Brain
Kidney
Liver
Prostate

Associated OMIM IDs

114500 (Colorectal cancer)
610247 (Eosinophilic esophagitis)

Human Proteins and Enzymes

Protein ID	Protein Name	Protein Type	Uniprot ID	Species
MMDBp0192186	Cystathionine gamma-lyase	Enzyme	P32929	Homo sapiens
MMDBp0192399	Cystathionine beta-synthase	Unknown	P35520	Homo sapiens

Human Pathways

Pathways

Name	SMPDB/PathBank

Metabolic Reactions

Reactions

Reaction ID	Precursor	Product	Enzyme	Data Source	Reference
MMDBr0004196	L-Cystathionine	N-Acetyl-L-Cystathionine	N-acetylglutamate synthase	VMH	7126172
MMDBr0007056	L-Cystathionine	Homocysteine	Not Available	VMH	30371894
MMDBr0007125	O-Acetyl-L-homoserine	Acetic acid	Not Available	VMH	30371894
MMDBr0007143	L-Cysteine	L-Cystathionine	Not Available	VMH	30371894
MMDBr0007293	L-Cystathionine	2-Ketobutyric acid	Not Available	VMH; KEGG	30371894
MMDBr0007294	Homocysteine	L-Cystathionine	Not Available	VMH; KEGG	30371894
MMDBr0010952	L-Cysteine	L-Cystathionine	Not Available	VMH	30371894
MMDBr0012690	L-Cysteine	L-Cystathionine	Not Available	VMH	30371894

Health Effects and Bioactivity

Health Outcome/Bioactivity	Metabolite Response/Effect	Evidence Type	Host and Biospecimen	Data Source	Reference
Alzheimer's disease		Association	Human Blood	HMDB	17031479
Alzheimer's disease	Decreased	Association	Human Urine	HMDB	17031479
Autism		Association	Human Blood	HMDB	15585776
Cystathioninuria		Association	Human Urine	HMDB	20584029
Cystathioninuria		Association	Human Blood	HMDB	20584029
Cystathioninuria	Increased	Association	Human Urine	HMDB	1429979
Hypermethioninemia		Association	Human Blood	HMDB	15024124
Vitamin b12 deficiency	Slight Increase	Association	Human Blood	HMDB	8507876
Eosinophilic esophagitis		Association	Human Urine	HMDB	Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work)
Cystathioninuria		Association	Human Urine	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)
Cystathioninuria	Slight Decrease	Association	Human Urine	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)

Microbial Sources

Superkingdom/Kingdom	Phylum	Total species	Hosts and Body Sites	Microbial Links
Bacteria	Bacteroidetes	119	Human ; Human feces; Human appendix biopsy; Human stool; Human saliva; Human supragingival plaque; Human subgingival plaque; Human gastric fluid; Human urine	Indirect association between microbiota composition and metabolites measured in host biospecimen; Metabolic reconstruction
Bacteria	Proteobacteria	173	Human stool; Human ; Human feces; Human sputum; Human unknown; Pig ; Human pleural fluid plaque; Human dental plaque; Human nasal swab; Human feces; pleural fluid plaque; bal	Indirect association between microbiota composition and metabolites measured in host biospecimen; Metabolic reconstruction
Bacteria	Firmicutes	310	Human feces; Human stool; Human ; Human pleural fluid plaque; Pig ; Human sputum; Human subgingival plaque; Human saliva; Human urine; Human supragingival plaque; Cattle	Indirect association between microbiota composition and metabolites measured in host biospecimen; Metabolic reconstruction
Bacteria	Actinobacteria	121	Human feces; Human sputum; Human bronchial brush; Human nasopharyngeal swab; Human pleural fluid plaque; Human dental plaque; Human ; Human mucosa; Human saliva; Human supragingival plaque; Human subgingival plaque; Cattle	Metabolic reconstruction
Bacteria	Verrucomicrobia	1	Human ; Human feces	Metabolic reconstruction
Archaea	Thaumarchaeota	1		Metabolic reconstruction
Bacteria	Fusobacteria	9	Human ; Human feces	Metabolic reconstruction
Archaea	Euryarchaeota	4		Metabolic reconstruction
Bacteria	Candidatus melainabacteria	1	Human feces	Metabolic reconstruction
Bacteria	Synergistetes	1	Human feces	Metabolic reconstruction
Bacteria	Planctomycetes	1	Human feces	Metabolic reconstruction
Archaea	Crenarchaeota	1		Metabolic reconstruction
Bacteria	Tenericutes	2	Human feces	Metabolic reconstruction
Eukaryota/Fungi	Ascomycota	4	Human feces; Human saliva	Unknown
Bacteria	Deinococcus-thermus	1
Eukaryota/Fungi	Basidiomycota	1
Bacteria	Thermotogae	1
Archaea/Archaea	Crenarchaeota	1		Metabolic reconstruction
Bacteria	Chloroflexi	1

Exposure Sources

Source Type	Source Sub-type	Species	Data Source	Reference

Host Biospecimen and Location

Host and Biospecimen	Status	Mean	Std	Min	Max	Units	Age	Sex	Health Condition	Data Source	Reference
Human Feces	Detected but not Quantified									HMDB
Human Blood	Detected and Quantified	1.03	0.22			uM	Elderly (>65 years old)	Both	Alzheimer's disease	HMDB	17031479
Human Urine	Detected and Quantified	1.68	0.5			umol/mmol creatinine	Adult (>18 years old)	Both	Alzheimer's disease	HMDB	17031479
Human Blood	Detected and Quantified	0.14		0.04	0.2	uM	Children (1-13 years old)	Both	Autism	HMDB	15585776
Human Urine	Detected and Quantified	6.139				umol/mmol creatinine	Children (1-13 years old)	Female	Cystathioninuria	HMDB	20584029
Human Urine	Detected and Quantified	6.897				umol/mmol creatinine	Children (1-13 years old)	Female	Cystathioninuria	HMDB	20584029
Human Blood	Detected and Quantified	10				uM	Children (1-13 years old)	Female	Cystathioninuria	HMDB	20584029
Human Urine	Detected and Quantified	84.795				umol/mmol creatinine	Children (1-13 years old)	Female	Cystathioninuria	HMDB	20584029
Human Urine	Detected and Quantified	182.14				umol/mmol creatinine	Children (1-13 years old)	Female	Cystathioninuria	HMDB	20584029
Human Urine	Detected and Quantified	216.623				umol/mmol creatinine	Children (1-13 years old)	Female	Cystathioninuria	HMDB	20584029
Human Urine	Detected and Quantified	381.0	29.0			umol/mmol creatinine	Adult (>18 years old)	Both	Cystathioninuria	HMDB	1429979
Human Blood	Detected and Quantified	1.56		0.14	4.1	uM	Adult (>18 years old)	Both	Folate Deficiency	HMDB	8507876
Human Blood	Detected and Quantified	0.552				uM	Children (1-13 years old)	Male	Hypermethioninemia	HMDB	15024124
Human Blood	Detected and Quantified	0.82		0.21	2.9	uM	Adult (>18 years old)	Both	Cobalamin Deficiency	HMDB	8507876
Human Urine	Detected and Quantified			0	17	umol/mmol creatinine	Infant (0-1 year old)	Both	Normal	HMDB	30830347
Human Cerebrospinal fluid (csf)	Detected and Quantified	0.024	0.006			uM	Children (1-13 years old)	Both	Normal	HMDB	17409006
Human Blood	Detected and Quantified			0.0440	0.342	uM	Adult (>18 years old)	Not Specified	Normal	HMDB	15024124
Human Blood	Detected and Quantified	0.126		0.065	0.301	uM	Adult (>18 years old)	Both	Normal	HMDB	8507876
Human Blood	Detected and Quantified	0.17	0.1 ‚Äì 0.27			uM	Children (1-13 years old)	Both	Normal	HMDB	15585776
Human Blood	Detected and Quantified	0.329		0.150	0.661	uM	Adult (>18 years old)	Both	Normal	HMDB	17384003
Human Urine	Detected and Quantified	0.35	0.65			umol/mmol creatinine	Infant (0-1 year old)	Both	Normal	HMDB	2026685
Human Urine	Detected and Quantified			0.80	1.6	umol/mmol creatinine	Adult (>18 years old)	Male	Normal	HMDB	27012787
Human Urine	Detected and Quantified			0.94	2.3	umol/mmol creatinine	Adult (>18 years old)	Female	Normal	HMDB	27012787
Human Urine	Detected and Quantified	1.54 ¬± 1.09				umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	9367221
Human Urine	Detected and Quantified	14.7		3.9	20.7	umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	24023812
Human Urine	Detected and Quantified	32.79	27.13			umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	HMDB	19468821
Human Urine	Detected and Quantified	36.18	35.05			umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	HMDB	19468821
Human Urine	Detected and Quantified	41.83	32.79			umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	HMDB	19468821
Human Urine	Detected and Quantified	12.677	3.531			umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Eosinophilic esophagitis	HMDB	Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work)
Human Urine	Detected and Quantified	1001.0		158.0	1843.8	umol/mmol creatinine	Children (1-13 years old)	Both	Gamma-Cystathionase deficiency	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)
Human Urine	Detected and Quantified	5.3		1.0	9.6	umol/mmol creatinine	Adult (>18 years old)	Both	Cystathioninuria	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)
Human Blood	Detected and Quantified	1.0		0.3	5.5	uM	Newborn (0-30 days old)	Both	Normal	HMDB	Geigy Scientific Tables, 8th Rev edition, pp. 130. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp. Basel, Switzerland c1981-1992.
Human Urine	Detected and Quantified	<2.036				umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	HMDB	Mayo Clinic (https://neurology.testcatalog.org/show/AAPD)
Human Urine	Detected and Quantified	<2.94				umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	HMDB	Mayo Clinic (https://neurology.testcatalog.org/show/AAPD)
Human Urine	Detected and Quantified	<3.39				umol/mmol creatinine	Adult (>18 years old)	Not Specified	Normal	HMDB	Mayo Clinic (https://neurology.testcatalog.org/show/AAPD)
Human Urine	Detected and Quantified	<34.16				umol/mmol creatinine	Infant (0-1 year old)	Not Specified	Normal	HMDB	Mayo Clinic (https://neurology.testcatalog.org/show/AAPD)
Human Urine	Detected and Quantified	<4.98				umol/mmol creatinine	Adolescent (13-18 years old)	Not Specified	Normal	HMDB	Mayo Clinic (https://neurology.testcatalog.org/show/AAPD)
Human Urine	Detected and Quantified	<6.33				umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	HMDB	Mayo Clinic (https://neurology.testcatalog.org/show/AAPD)
Human Brain	Detected but not Quantified						Not Specified	Not Specified	Normal	HMDB	34986597
Human Kidney	Detected but not Quantified						Not Specified	Not Specified	Normal	HMDB	34986597
Human Liver	Detected but not Quantified						Not Specified	Not Specified	Normal	HMDB	34986597
Human Prostate	Detected but not Quantified						Not Specified	Not Specified	Normal	HMDB	34986597
Human Feces	Detected but not Quantified						Children (6 - 18 years old)	Not Specified	Normal	HMDB	27609529
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	29808030

External Links

HMDB ID

HMDB0000099

DrugBank ID

Not Available

Phenol Explorer Compound ID

Not Available

FooDB ID

KNApSAcK ID

Chemspider ID

KEGG Compound ID

BioCyc ID

BiGG ID

Wikipedia Link

METLIN ID

PubChem Compound

439258

PDB ID

Not Available

ChEBI ID

17482

Food Biomarker Ontology

Not Available

References

Synthesis Reference

Not Available

General References

Lefauconnier JM, Portemer C, Chatagner F: Free amino acids and related substances in human glial tumours and in fetal brain: comparison with normal adult brain. Brain Res. 1976 Nov 19;117(1):105-13. doi: 10.1016/0006-8993(76)90559-x. [PubMed:186155 ]
Haurani FI, Hall CA, Rubin R: Megaloblastic anemia as a result of an abnormal transcobalamin II (Cardeza). J Clin Invest. 1979 Nov;64(5):1253-9. doi: 10.1172/JCI109580. [PubMed:500809 ]
Shoemaker JD, Elliott WH: Automated screening of urine samples for carbohydrates, organic and amino acids after treatment with urease. J Chromatogr. 1991 Jan 2;562(1-2):125-38. doi: 10.1016/0378-4347(91)80571-s. [PubMed:2026685 ]
Wisniewski K, Sturman JA, Devine E, Brown WT, Rudelli R, Wisniewski HM: Cystathionine disappearance with neuronal loss: a possible neuronal marker. Neuropediatrics. 1985 Aug;16(3):126-30. doi: 10.1055/s-2008-1052556. [PubMed:4047345 ]
Shimizu H, Kakimoto Y, Sano I: A method of determination of cystathionine and its distribution in human brain. J Neurochem. 1966 Feb;13(2):65-73. doi: 10.1111/j.1471-4159.1966.tb03333.x. [PubMed:5936154 ]
Ricci G, Santoro L, Achilli M, Matarese RM, Nardini M, Cavallini D: Similarity of the oxidation products of L-cystathionine by L-amino acid oxidase to those excreted by cystathioninuric patients. J Biol Chem. 1983 Sep 10;258(17):10511-7. [PubMed:6885789 ]
Stabler SP, Lindenbaum J, Savage DG, Allen RH: Elevation of serum cystathionine levels in patients with cobalamin and folate deficiency. Blood. 1993 Jun 15;81(12):3404-13. [PubMed:8507876 ]
Kraus JP, Janosik M, Kozich V, Mandell R, Shih V, Sperandeo MP, Sebastio G, de Franchis R, Andria G, Kluijtmans LA, Blom H, Boers GH, Gordon RB, Kamoun P, Tsai MY, Kruger WD, Koch HG, Ohura T, Gaustadnes M: Cystathionine beta-synthase mutations in homocystinuria. Hum Mutat. 1999;13(5):362-75. doi: 10.1002/(SICI)1098-1004(1999)13:5<362::AID-HUMU4>3.0.CO;2-K. [PubMed:10338090 ]
Herrmann W, Schorr H, Bodis M, Knapp JP, Muller A, Stein G, Geisel J: Role of homocysteine, cystathionine and methylmalonic acid measurement for diagnosis of vitamin deficiency in high-aged subjects. Eur J Clin Invest. 2000 Dec;30(12):1083-9. doi: 10.1046/j.1365-2362.2000.00746.x. [PubMed:11122323 ]
James SJ, Cutler P, Melnyk S, Jernigan S, Janak L, Gaylor DW, Neubrander JA: Metabolic biomarkers of increased oxidative stress and impaired methylation capacity in children with autism. Am J Clin Nutr. 2004 Dec;80(6):1611-7. doi: 10.1093/ajcn/80.6.1611. [PubMed:15585776 ]
Sreekumar A, Poisson LM, Rajendiran TM, Khan AP, Cao Q, Yu J, Laxman B, Mehra R, Lonigro RJ, Li Y, Nyati MK, Ahsan A, Kalyana-Sundaram S, Han B, Cao X, Byun J, Omenn GS, Ghosh D, Pennathur S, Alexander DC, Berger A, Shuster JR, Wei JT, Varambally S, Beecher C, Chinnaiyan AM: Metabolomic profiles delineate potential role for sarcosine in prostate cancer progression. Nature. 2009 Feb 12;457(7231):910-4. doi: 10.1038/nature07762. [PubMed:19212411 ]

Showing metabocard for L-Cystathionine (MMDBc0000495)

MOL for #<Metabolite:0x00007fec9d126cb8>

3D MOL for #<Metabolite:0x00007fec9d126cb8>

3D SDF for #<Metabolite:0x00007fec9d126cb8>

3D-SDF for #<Metabolite:0x00007fec9d126cb8>

PDB for #<Metabolite:0x00007fec9d126cb8>

3D PDB for #<Metabolite:0x00007fec9d126cb8>

SMILES for #<Metabolite:0x00007fec9d126cb8>

INCHI for #<Metabolite:0x00007fec9d126cb8>

Structure for #<Metabolite:0x00007fec9d126cb8>

3D Structure for #<Metabolite:0x00007fec9d126cb8>