DrugBank: L-Cystine (DB00138)

targets (4) transporters (1)

Identification

Name

L-Cystine

Accession Number

DB00138 (NUTR00023)

Type

small molecule

Groups

approved, nutraceutical

Description

A covalently linked dimeric nonessential amino acid formed by the oxidation of cysteine. Two molecules of cysteine are joined together by a disulfide bridge to form cystine. [PubChem]

Structure

Download: MOL | SDF | SMILES | InChI
Display: 2D Structure | 3D Structure

Synonyms

(-)-Cystine
Cysteine disulfide
Cystine acid
L-alpha-Diamino-beta-dithiolactic acid
L-Dicysteine

Brand names

Not Available

Brand name mixtures

Not Available

Categories

Dietary supplement
Micronutrient
Non-Essential Amino Acids

CAS number

56-89-3

Weight

Average: 240.3
Monoisotopic: 240.023848262

Chemical Formula

C₆H₁₂N₂O₄S₂

InChI Key

InChIKey=LEVWYRKDKASIDU-IMJSIDKUSA-N

InChI

InChI=1S/C6H12N2O4S2/c7-3(5(9)10)1-13-14-2-4(8)6(11)12/h3-4H,1-2,7-8H2,(H,9,10)(H,11,12)/t3-,4-/m0/s1

Plain Text

IUPAC Name

(2R)-2-amino-3-{[(2R)-2-amino-2-carboxyethyl]disulfanyl}propanoic acid

SMILES

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

Plain Text

Mass Spec

show (3 KB)

Taxonomy

Kingdom

Organic

Classes

Amino Acids

Substructures

Amino Acids
Hydroxy Compounds
Acetates
Aliphatic and Aryl Amines
Organic Disulfides
Carboxylic Acids and Derivatives

Pharmacology

Indication

It has been claimed that L-cysteine has anti-inflammatory properties, that it can protect against various toxins, and that it might be helpful in osteoarthritis and rheumatoid arthritis. More research will have to be done before L-cysteine can be indicated for any of these conditions. Research to date has mostly been in animal models.

Pharmacodynamics

L-Cystine is a covalently linked dimeric nonessential amino acid formed by the oxidation of cysteine. Two molecules of cysteine are joined together by a disulfide bridge to form cystine. Cystine is a chemical substance which naturally occurs as a deposit in the urine, and can form a calculus (hard mineral formation) when deposited in the kidney. The compound produced when two cysteine molecules linked by a disulfide (S-S) bond. Cystine is required for proper vitamin B6 utilization and is also helpful in the healing of burns and wounds, breaking down mucus deposits in illnesses such as bronchitis as well as cystic fibrosis. Cysteine also assists in the supply of insulin to the pancreas, which is needed for the assimilation of sugars and starches. It increases the level of glutathione in the lungs, liver, kidneys and bone marrow, and this may have an anti-aging effect on the body by reducing age-spots etc.

Mechanism of action

Certain conditions, e.g. an acetaminophen overdose, deplete hepatic glutathione and subject the tissues to oxidative stress resulting in loss of cellular integrity. L-Cystine serves as a major precursor for synthesis of glutathione.

Absorption

Not Available

Volume of distribution

Not Available

Protein binding

Not Available

Metabolism

Route of elimination

Not Available

Half life

Not Available

Clearance

Not Available

Toxicity

With typical doses of 1 to 1.5 grams daily, the most commonly reported side effects have been gastrointestinal, such as nausea. There are rare reports of cystine renal stone formation, Single injections of L-cysteine (0.6-1.5 g/kg) into 4-day-old pups resulted in massive damage to cortical neurons, permanent retinal dystrophy, atrophy of the brain and hyperactivity.

Affected organisms

Humans and other mammals

Pathways

Not Available

Pharmacoeconomics

Manufacturers

Not Available

Packagers

Mason Distributors
Tyson & Associates Inc.

Dosage forms

Not Available

Prices

Unit description	Cost	Unit
L-alanine 600 mg capsule	0.35 USD	capsule
A-ketoglut acid 500 mg capsule	0.31 USD	capsule
Amino acid capsule	0.08 USD	capsule

Patents

Not Available

Properties

State

solid

Melting point

260-261 oC

Experimental Properties

Property	Value	Source
water solubility	Soluble	PhysProp
logP	-5.5	PhysProp

Predicted Properties

Property	Value	Source
water solubility	1.68e+01 g/l	ALOGPS
logP	-3.16	ALOGPS
logP	-6.17	ChemAxon Molconvert
logS	-1.16	ALOGPS
pKa	2.27	ChemAxon Molconvert
hydrogen acceptor count	6	ChemAxon Molconvert
hydrogen donor count	4	ChemAxon Molconvert
polar surface area	126.64	ChemAxon Molconvert
rotatable bond count	7	ChemAxon Molconvert
refractivity	54.87	ChemAxon Molconvert
polarizability	22.67	ChemAxon Molconvert

References

Synthesis Reference

Not Available

General Reference

Not Available

External Links

Resource	Link
KEGG Drug	D03636
KEGG Compound	C00491
PubChem Compound	67678
PubChem Substance	46506670
ChemSpider	60997
ChEBI	16283
ChEMBL	16283
PDRhealth	http://www.pdrhealth.com/drug_info/nmdrugprofiles/nutsupdrugs/lcy_0088.shtml

ATC Codes

Not Available

AHFS Codes

Not Available

PDB Entries

1SSQ

FDA label

Not Available

MSDS

show (72 KB)

Interactions

Drug Interactions

Drug	Interaction

Food Interactions

Not Available

Targets
1. Cystine/glutamate transporter Pharmacological action: unknown Sodium-independent, high-affinity exchange of anionic amino acids with high specificity for anionic form of cystine and glutamate Organism class: human UniProt ID: Q9UPY5 Gene: SLC7A11 Protein Sequence: FASTA Gene Sequence: FASTA SNPs: SNPJam Report References: Tomi M, Hosoya K, Takanaga H, Ohtsuki S, Terasaki T: Induction of xCT gene expression and L-cystine transport activity by diethyl maleate at the inner blood-retinal barrier. Invest Ophthalmol Vis Sci. 2002 Mar;43(3):774-9. Pubmed Hosoya K, Tomi M, Ohtsuki S, Takanaga H, Saeki S, Kanai Y, Endou H, Naito M, Tsuruo T, Terasaki T: Enhancement of L-cystine transport activity and its relation to xCT gene induction at the blood-brain barrier by diethyl maleate treatment. J Pharmacol Exp Ther. 2002 Jul;302(1):225-31. Pubmed Ruiz E, Siow RC, Bartlett SR, Jenner AM, Sato H, Bannai S, Mann GE: Vitamin C inhibits diethylmaleate-induced L-cystine transport in human vascular smooth muscle cells. Free Radic Biol Med. 2003 Jan 1;34(1):103-10. Pubmed Bridges CC, Zalups RK: Cystine and glutamate transport in renal epithelial cells transfected with human system x(-) ©. Kidney Int. 2005 Aug;68(2):653-64. Pubmed Tomi M, Funaki T, Abukawa H, Katayama K, Kondo T, Ohtsuki S, Ueda M, Obinata M, Terasaki T, Hosoya K: Expression and regulation of L-cystine transporter, system xc-, in the newly developed rat retinal Muller cell line (TR-MUL). Glia. 2003 Sep;43(3):208-17. Pubmed 2. Cystinosin Pharmacological action: unknown Thought to transport cystine out of lysosomes Organism class: human UniProt ID: O60931 Gene: CTNS Protein Sequence: FASTA Gene Sequence: FASTA SNPs: SNPJam Report References: Kalatzis V, Cherqui S, Antignac C, Gasnier B: Cystinosin, the protein defective in cystinosis, is a H(+)-driven lysosomal cystine transporter. EMBO J. 2001 Nov 1;20(21):5940-9. Pubmed 3. B(0,+)-type amino acid transporter 1 Pharmacological action: unknown Involved in the high-affinity, sodium-independent transport of cystine and neutral and dibasic amino acids (system b(0,+)-like activity). Thought to be responsible for the high- affinity reabsorption of cystine in the kidney tubule Organism class: human UniProt ID: P82251 Gene: SLC7A9 Protein Sequence: FASTA Gene Sequence: FASTA SNPs: SNPJam Report References: Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed 4. Neutral and basic amino acid transport protein rBAT Pharmacological action: unknown Involved in the high-affinity, sodium-independent transport of cystine and neutral and dibasic amino acids (system B(0,+)-like activity). May function as an activator of SLC7A9 and be involved in the high-affinity reabsorption of cystine in the kidney tubule Organism class: human UniProt ID: Q07837 Gene: SLC3A1 Protein Sequence: FASTA Gene Sequence: FASTA SNPs: SNPJam Report References: Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed

Targets

1. Cystine/glutamate transporter

Pharmacological action: unknown

Sodium-independent, high-affinity exchange of anionic amino acids with high specificity for anionic form of cystine and glutamate

Organism class: human
UniProt ID: Q9UPY5 Link_out

Gene: SLC7A11 Link_out

Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report Link_out

References:

Tomi M, Hosoya K, Takanaga H, Ohtsuki S, Terasaki T: Induction of xCT gene expression and L-cystine transport activity by diethyl maleate at the inner blood-retinal barrier. Invest Ophthalmol Vis Sci. 2002 Mar;43(3):774-9. Pubmed
Hosoya K, Tomi M, Ohtsuki S, Takanaga H, Saeki S, Kanai Y, Endou H, Naito M, Tsuruo T, Terasaki T: Enhancement of L-cystine transport activity and its relation to xCT gene induction at the blood-brain barrier by diethyl maleate treatment. J Pharmacol Exp Ther. 2002 Jul;302(1):225-31. Pubmed
Ruiz E, Siow RC, Bartlett SR, Jenner AM, Sato H, Bannai S, Mann GE: Vitamin C inhibits diethylmaleate-induced L-cystine transport in human vascular smooth muscle cells. Free Radic Biol Med. 2003 Jan 1;34(1):103-10. Pubmed
Bridges CC, Zalups RK: Cystine and glutamate transport in renal epithelial cells transfected with human system x(-) ©. Kidney Int. 2005 Aug;68(2):653-64. Pubmed
Tomi M, Funaki T, Abukawa H, Katayama K, Kondo T, Ohtsuki S, Ueda M, Obinata M, Terasaki T, Hosoya K: Expression and regulation of L-cystine transporter, system xc-, in the newly developed rat retinal Muller cell line (TR-MUL). Glia. 2003 Sep;43(3):208-17. Pubmed

2. Cystinosin

Pharmacological action: unknown

Thought to transport cystine out of lysosomes

Organism class: human
UniProt ID: O60931 Link_out

Gene: CTNS Link_out

Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report Link_out

References:

Kalatzis V, Cherqui S, Antignac C, Gasnier B: Cystinosin, the protein defective in cystinosis, is a H(+)-driven lysosomal cystine transporter. EMBO J. 2001 Nov 1;20(21):5940-9. Pubmed

3. B(0,+)-type amino acid transporter 1

Pharmacological action: unknown

Involved in the high-affinity, sodium-independent transport of cystine and neutral and dibasic amino acids (system b(0,+)-like activity). Thought to be responsible for the high- affinity reabsorption of cystine in the kidney tubule

Organism class: human
UniProt ID: P82251 Link_out

Gene: SLC7A9 Link_out

Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report Link_out

References:

Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed
Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed

4. Neutral and basic amino acid transport protein rBAT

Pharmacological action: unknown

Involved in the high-affinity, sodium-independent transport of cystine and neutral and dibasic amino acids (system B(0,+)-like activity). May function as an activator of SLC7A9 and be involved in the high-affinity reabsorption of cystine in the kidney tubule

Organism class: human
UniProt ID: Q07837 Link_out

Gene: SLC3A1 Link_out

Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report Link_out

References:

Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed
Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed

Transporters
1. Monocarboxylate transporter 10 Actions: inhibitor Sodium-independent transporter that mediates the update of aromatic acid. Can function as a net efflux pathway for aromatic amino acids in the basosolateral epithelial cells (By similarity) UniProt ID: Q8TF71 Gene: SLC16A10 Protein Sequence: FASTA Gene Sequence: FASTA SNPs: SNPJam Report References: Kim DK, Kanai Y, Chairoungdua A, Matsuo H, Cha SH, Endou H: Expression cloning of a Na+-independent aromatic amino acid transporter with structural similarity to H+/monocarboxylate transporters. J Biol Chem. 2001 May 18;276(20):17221-8. Epub 2001 Feb 20. Pubmed

Transporters

1. Monocarboxylate transporter 10

Actions: inhibitor

Sodium-independent transporter that mediates the update of aromatic acid. Can function as a net efflux pathway for aromatic amino acids in the basosolateral epithelial cells (By similarity)

UniProt ID: Q8TF71 Link_out

Gene: SLC16A10 Link_out

Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report Link_out

References:

Kim DK, Kanai Y, Chairoungdua A, Matsuo H, Cha SH, Endou H: Expression cloning of a Na+-independent aromatic amino acid transporter with structural similarity to H+/monocarboxylate transporters. J Biol Chem. 2001 May 18;276(20):17221-8. Epub 2001 Feb 20. Pubmed

Comments

Drug created on June 13, 2005 07:24 / Updated on November 10, 2010 13:36

This project is supported by Genome Alberta & Genome Canada, a not-for-profit organization that is leading Canada's national genomics strategy with $600 million in funding from the federal government. This project is also supported in part by GenomeQuest, Inc., an enterprise genomic information company serving the life science community.