Human Metabolome Database: Showing metabocard for L-Cystathionine (HMDB0000099)

You are using an unsupported browser. Please upgrade your browser to a newer version to get the best experience on Human Metabolome Database.

Jump To Section:

Identification Taxonomy Ontology Physical properties Spectra Biological properties Concentrations Links References enzymes (2) Show 2 proteins XML

enzymes (2) Show 2 proteins

Record Information

Version

4.0

Status

Detected and Quantified

Creation Date

2005-11-16 15:48:42 UTC

Update Date

2020-03-25 20:10:11 UTC

HMDB ID

HMDB0000099

Secondary Accession Numbers

HMDB0003614
HMDB00099
HMDB03614

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 SDF 3D-SDF PDB SMILES InChI View 3D Structure

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

Chemical Formula

C₇H₁₄N₂O₄S

Average Molecular Weight

222.262

Monoisotopic Molecular Weight

222.067427636

IUPAC Name

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

Traditional Name

L-cystathionine

CAS Registry Number

56-88-2

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 )

Ontology

Ingestion

Source:

Biological:

Plant:

Animal

Biological location:

Organ and components:

Biofluid and excreta:

Subcellular:

Cytoplasm

Process

Naturally occurring process:

Physical Properties

State

Solid

Experimental Properties

Property	Value	Reference
Melting Point	312 °C	Not Available
Boiling Point	Not Available	Not Available
Water Solubility	22 mg/mL	Not Available
LogP	Not Available	Not Available

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	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	Spectrum
GC-MS	GC-MS Spectrum - GC-MS (2 TMS)	splash10-0f7c-7910000000-91f9f2b2dd44a3cdb276	Spectrum
GC-MS	GC-MS Spectrum - GC-MS (3 TMS)	splash10-00or-1940000000-c0d688e4ea87ff3e03fa	Spectrum
GC-MS	GC-MS Spectrum - GC-MS (4 TMS)	splash10-00or-0980000000-7d816de782a902900d0e	Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-00fr-8950000000-640b4294e37e5a01048d	Spectrum
GC-MS	GC-MS Spectrum - GC-MS (Non-derivatized)	splash10-0f7c-7910000000-91f9f2b2dd44a3cdb276	Spectrum
GC-MS	GC-MS Spectrum - GC-MS (Non-derivatized)	splash10-00or-1940000000-c0d688e4ea87ff3e03fa	Spectrum
GC-MS	GC-MS Spectrum - GC-MS (Non-derivatized)	splash10-00or-0980000000-7d816de782a902900d0e	Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive	splash10-00di-9410000000-9487e9168e86a2297931	Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (2 TMS) - 70eV, Positive	splash10-00dm-9665000000-608a6335880ad1c2519d	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)	splash10-0089-1980000000-99cb7d1da067e5436b09	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)	splash10-000i-9200000000-fb776970b6ef36e72725	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)	splash10-000i-9200000000-fb776970b6ef36e72725	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Positive	splash10-00fr-0690000000-0b572de3fcbc7e2f13cc	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Positive	splash10-000t-0900000000-5788192b32aadd177e33	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Positive	splash10-000b-4900000000-b86ac7de446a762365bd	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Positive	splash10-000j-9800000000-75ff1bc6e325692a9da9	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Positive	splash10-00kr-9100000000-6517562dc0f31a85afc8	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-IT (LC/MSD Trap XCT, Agilent Technologies) , Positive	splash10-001i-0900000000-d1628d0a880f1b6e6d90	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-IT (LC/MSD Trap XCT, Agilent Technologies) , Positive	splash10-000i-9000000000-9a2e1e54b77216e2df72	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Positive	splash10-00di-0090000000-36678c2f0bf1b01cc9d4	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) 30V, Positive	splash10-05fr-0190000000-e575f57f0d76ef6afa49	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Negative	splash10-00e9-0940000000-962ae510f06296b98878	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QTOF , negative	splash10-00e9-0940000000-27da1912eac54f485bfa	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ , positive	splash10-00fr-0690000000-82fe2198c98660008c04	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ , positive	splash10-000t-0900000000-58cceb69cceeff79606c	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ , positive	splash10-000b-4900000000-5877787c54120dc165c9	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ , positive	splash10-000j-9800000000-fd29b0fcf62b73831829	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ , positive	splash10-00kr-9100000000-6517562dc0f31a85afc8	Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Positive	splash10-05di-1930000000-887e3ad7da04e4f1aac9	Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Positive	splash10-01uc-6900000000-563444ac8ab717997268	Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Positive	splash10-0uki-9700000000-35397d8e2ab2c6582c52	Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Negative	splash10-00di-0970000000-d0f0a96ca41d1f751c26	Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Negative	splash10-0089-3910000000-22200c974305822456d8	Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Negative	splash10-008l-9300000000-0dfaab5efe3438889c3c	Spectrum
1D NMR	1H NMR Spectrum	Not Available	Spectrum
1D NMR	1H NMR Spectrum	Not Available	Spectrum
1D NMR	13C NMR Spectrum	Not Available	Spectrum
2D NMR	[1H,13C] 2D NMR Spectrum	Not Available	Spectrum

Biological Properties

Cellular Locations

Cytoplasm (predicted from logP)

Biospecimen Locations

Blood
Cerebrospinal Fluid (CSF)
Feces
Urine

Tissue Locations

Brain
Kidney
Liver
Prostate

Pathways

Name	SMPDB/Pathwhiz	KEGG
Glycine and Serine Metabolism
Methionine Metabolism
Homocysteine Degradation		Not Available
Gamma-cystathionase deficiency (CTH)		Not Available
Homocystinuria, cystathionine beta-synthase deficiency		Not Available

Normal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Detected and Quantified	0.329 (0.150-0.661) uM	Adult (>18 years old)	Both	Normal	17384003	details
Blood	Detected and Quantified	0.0440-0.342 uM	Adult (>18 years old)	Not Specified	Normal	15024124	details
Blood	Detected and Quantified	0.17 (0.1 – 0.27) uM	Children (1-13 years old)	Both	Normal	15585776	details
Blood	Detected and Quantified	1.0 (0.3-5.5) uM	Newborn (0-30 days old)	Both	Normal	Geigy Scientific ...	details
Blood	Detected and Quantified	0.126 (0.065 - 0.301) uM	Adult (>18 years old)	Both	Normal	8507876	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	0.024 +/- 0.006 uM	Children (1-13 years old)	Both	Normal	17409006	details
Feces	Detected but not Quantified		Children (6 - 18 years old)	Not Specified	Normal	27609529	details
Feces	Detected but not Quantified		Adult (>18 years old)	Both	Normal	29808030	details
Urine	Detected and Quantified	1.84 (0.39-3.28) umol/mmol creatinine	Adult (>18 years old)	Both	Normal		details
Urine	Detected and Quantified	<2.036 umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	Mayo Clinic	details
Urine	Detected and Quantified	<4.98 umol/mmol creatinine	Adolescent (13-18 years old)	Not Specified	Normal	Mayo Clinic	details
Urine	Detected and Quantified	0.94-2.3 umol/mmol creatinine	Adult (>18 years old)	Female	Normal	27012787	details
Urine	Detected and Quantified	14.7 (3.9-20.7) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	24023812	details
Urine	Detected and Quantified	<3.39 umol/mmol creatinine	Adult (>18 years old)	Not Specified	Normal	Mayo Clinic	details
Urine	Detected and Quantified	<34.16 umol/mmol creatinine	Infant (0-1 year old)	Not Specified	Normal	Mayo Clinic	details
Urine	Detected and Quantified	0.80-1.6 umol/mmol creatinine	Adult (>18 years old)	Male	Normal	27012787	details
Urine	Detected and Quantified	<6.33 umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	Mayo Clinic	details
Urine	Detected and Quantified	0-17 umol/mmol creatinine	Infant (0-1 year old)	Both	Normal	30830347	details
Urine	Detected and Quantified	32.79 +/- 27.13 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	19468821	details
Urine	Detected and Quantified	36.18 +/- 35.05 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	19468821	details
Urine	Detected and Quantified	41.83 +/- 32.79 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	19468821	details
Urine	Detected and Quantified	1.54 ± 1.09 umol/mmol creatinine	Adult (>18 years old)	Both	Normal	9367221	details
Urine	Detected and Quantified	<2.94 umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	Mayo Clinic	details
Urine	Detected and Quantified	0.35 +/- 0.65 umol/mmol creatinine	Infant (0-1 year old)	Both	Normal	2026685	details

Abnormal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Detected and Quantified	0.552 uM	Children (1-13 years old)	Male	Hypermethioninemia	15024124	details
Blood	Detected and Quantified	10 uM	Children (1-13 years old)	Female	Cystathioninuria	20584029	details
Blood	Detected and Quantified	1.56 (0.14 - 4.1) uM	Adult (>18 years old)	Both	Folate Deficiency	8507876	details
Blood	Detected and Quantified	0.82 (0.21 - 2.9) uM	Adult (>18 years old)	Both	Cobalamin Deficiency	8507876	details
Blood	Detected and Quantified	0.14 (0.04-0.2) uM	Children (1-13 years old)	Both	Autism	15585776	details
Blood	Detected and Quantified	1.03 +/- 0.22 uM	Elderly (>65 years old)	Both	Alzheimer's disease	17031479	details
Feces	Detected but not Quantified		Children (6 - 18 years old)	Not Specified	Crohns disease	27609529	details
Feces	Detected but not Quantified		Children (6 - 18 years old)	Not Specified	Ulcerative colitis	27609529	details
Feces	Detected but not Quantified		Children (6 - 18 years old)	Not Specified	Unclassified IBD	27609529	details
Urine	Detected and Quantified	6.139 umol/mmol creatinine	Children (1-13 years old)	Female	Cystathioninuria	20584029	details
Urine	Detected and Quantified	6.897 umol/mmol creatinine	Children (1-13 years old)	Female	Cystathioninuria	20584029	details
Urine	Detected and Quantified	182.14 umol/mmol creatinine	Children (1-13 years old)	Female	Cystathioninuria	20584029	details
Urine	Detected and Quantified	84.795 umol/mmol creatinine	Children (1-13 years old)	Female	Cystathioninuria	20584029	details
Urine	Detected and Quantified	216.623 umol/mmol creatinine	Children (1-13 years old)	Female	Cystathioninuria	20584029	details
Urine	Detected and Quantified	381.0 +/- 29.0 umol/mmol creatinine	Adult (>18 years old)	Both	Cystathioninuria	1429979	details
Urine	Detected and Quantified	5.3 (1.0-9.6) umol/mmol creatinine	Adult (>18 years old)	Both	Cystathioninuria	MetaGene: Metabol...	details
Urine	Detected and Quantified	1001.0 (158.0-1843.8) umol/mmol creatinine	Children (1-13 years old)	Both	Gamma-Cystathionase deficiency	MetaGene: Metabol...	details
Urine	Detected and Quantified	12.677 +/- 3.531 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Eosinophilic esophagitis	Mordechai, Hien, ...	details
Urine	Detected and Quantified	1.68 +/- 0.5 umol/mmol creatinine	Adult (>18 years old)	Both	Alzheimer's disease	17031479	details

Associated Disorders and Diseases

Disease References

Folate deficiency
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 ]
Vitamin B12 deficiency
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 ]
Alzheimer's disease
Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed:17031479 ]
Autism
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. [PubMed:15585776 ]
Hypermethioninemia
Baric I, Fumic K, Glenn B, Cuk M, Schulze A, Finkelstein JD, James SJ, Mejaski-Bosnjak V, Pazanin L, Pogribny IP, Rados M, Sarnavka V, Scukanec-Spoljar M, Allen RH, Stabler S, Uzelac L, Vugrek O, Wagner C, Zeisel S, Mudd SH: S-adenosylhomocysteine hydrolase deficiency in a human: a genetic disorder of methionine metabolism. Proc Natl Acad Sci U S A. 2004 Mar 23;101(12):4234-9. Epub 2004 Mar 15. [PubMed:15024124 ]
Crohn's disease
Kolho KL, Pessia A, Jaakkola T, de Vos WM, Velagapudi V: Faecal and Serum Metabolomics in Paediatric Inflammatory Bowel Disease. J Crohns Colitis. 2017 Mar 1;11(3):321-334. doi: 10.1093/ecco-jcc/jjw158. [PubMed:27609529 ]
Ulcerative colitis
Kolho KL, Pessia A, Jaakkola T, de Vos WM, Velagapudi V: Faecal and Serum Metabolomics in Paediatric Inflammatory Bowel Disease. J Crohns Colitis. 2017 Mar 1;11(3):321-334. doi: 10.1093/ecco-jcc/jjw158. [PubMed:27609529 ]
Cystathioninuria
Sugahara K, Ohta J, Takemura M, Kodama H: Determination of cystathionine and perhydro-1,4-thiazepine-3,5-dicarboxylic acid in the urine of a patient with cystathioninuria using column liquid chromatography-mass spectrometry. J Chromatogr. 1992 Sep 2;579(2):318-23. [PubMed:1429979 ] Espinos C, Garcia-Cazorla A, Martinez-Rubio D, Martinez-Martinez E, Vilaseca MA, Perez-Duenas B, Kozich V, Palau F, Artuch R: Ancient origin of the CTH alelle carrying the c.200C>T (p.T67I) variant in patients with cystathioninuria. Clin Genet. 2010 Dec;78(6):554-9. doi: 10.1111/j.1399-0004.2010.01431.x. [PubMed:20584029 ] G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Eosinophilic esophagitis
(). Mordechai, Hien, and David S. Wishart. .

Associated OMIM IDs

104300 (Alzheimer's disease)
209850 (Autism)
613752 (Hypermethioninemia)
266600 (Crohn's disease)
219500 (Cystathioninuria)
610247 (Eosinophilic esophagitis)

External Links

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

VMH ID

Not Available

References

Synthesis Reference

Yamagata, Shuzo; Akamatsu, Tsuyoshi; Iwama, Tomonori. Immobilization of Saccharomyces cerevisiae cystathionine gamma-lyase and application of the product to cystathionine synthesis. Applied and Environmental Microbiology (2004), 70(6), 3766-3768.

Material Safety Data Sheet (MSDS)

Download (PDF)

General References

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 ]
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. [PubMed:11122323 ]
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. [PubMed:2026685 ]
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 ]
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. [PubMed:186155 ]
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. [PubMed:15585776 ]
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. [PubMed:5936154 ]
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. [PubMed:10338090 ]
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. [PubMed:500809 ]
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. [PubMed:4047345 ]
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 ]

Enzymes

Enzyme Details

1. Cystathionine gamma-lyase

General function:: Involved in pyridoxal phosphate binding
Specific function:: Catalyzes the last step in the trans-sulfuration pathway from methionine to cysteine. Has broad substrate specificity. Converts cystathionine to cysteine, ammonia and 2-oxobutanoate. Converts two cysteine molecules to lanthionine and hydrogen sulfide. Can also accept homocysteine as substrate. Specificity depends on the levels of the endogenous substrates. Generates the endogenous signaling molecule hydrogen sulfide (H2S), and so contributes to the regulation of blood pressure. Acts as a cysteine-protein sulfhydrase by mediating sulfhydration of target proteins: sulfhydration consists of converting -SH groups into -SSH on specific cysteine residues of target proteins such as GAPDH, PTPN1 and NF-kappa-B subunit RELA, thereby regulating their function.
Gene Name:: CTH
Uniprot ID:: P32929
Molecular weight:: 41259.91

Reactions

L-Cystathionine + Water → L-Cysteine + Ammonia + 2-Ketobutyric acid	details

Enzyme Details

2. Cystathionine beta-synthase

General function:: Involved in cysteine biosynthetic process from serine
Specific function:: Only known pyridoxal phosphate-dependent enzyme that contains heme. Important regulator of hydrogen sulfide, especially in the brain, utilizing cysteine instead of serine to catalyze the formation of hydrogen sulfide. Hydrogen sulfide is a gastratransmitter with signaling and cytoprotective effects such as acting as a neuromodulator in the brain to protect neurons against hypoxic injury (By similarity).
Gene Name:: CBS
Uniprot ID:: P35520
Molecular weight:: 60586.05

Reactions

L-Serine + Homocysteine → L-Cystathionine + Water	details

Showing metabocard for L-Cystathionine (HMDB0000099)

Structure for HMDB0000099 (L-Cystathionine)

Enzymes

Reactions

Reactions