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Identification
NameCysteamine
Accession NumberDB00847  (APRD00896)
TypeSmall Molecule
GroupsApproved, Investigational
Description

Cysteamine is a radiation-protective agent that oxidizes in air to form cystamine. It can be given intravenously or orally to treat radiation sickness. The bitartrate and hydrochloride salt forms are indicated for the treatment of neuropathic cystinosis in patients 6 years old and older. [PubChem]. Cysteamine is marketed under several brand names such as Cystaran™, Procysbi, and Cystagon®.

Structure
Thumb
Synonyms
SynonymLanguageCode
2-Amino-1-ethanethiolNot AvailableNot Available
2-AMINO-ethanethiolNot AvailableNot Available
2-AminoethanethiolNot AvailableNot Available
beta-AminoethanethiolNot AvailableNot Available
beta-AminoethylthiolNot AvailableNot Available
beta-MEANot AvailableNot Available
beta-MercaptoethylamineNot AvailableNot Available
CysteamineNot AvailableNot Available
MEANot AvailableNot Available
MercaptaminaNot AvailableNot Available
MercaptamineNot AvailableNot Available
MercaptaminumNot AvailableNot Available
ThioethanolamineNot AvailableNot Available
β-aminoethylthiolNot AvailableNot Available
β-MEANot AvailableNot Available
Salts
Name/CAS Structure Properties
Cysteamine Bitartrate
Thumb
  • InChI Key: NSKJTUFFDRENDM-UHFFFAOYNA-N
  • Monoisotopic Mass: 227.046357843
  • Average Mass: 227.236
DBSALT000032
Cysteamine Hydrochloride
Thumb
  • InChI Key: OGMADIBCHLQMIP-UHFFFAOYSA-N
  • Monoisotopic Mass: 113.006597658
  • Average Mass: 113.61
DBSALT000033
Brand names
NameCompany
CystagonMylan
CystaranSigma Tau
ProcysbiRaptor Pharms
Brand mixturesNot Available
Categories
CAS number60-23-1
WeightAverage: 77.149
Monoisotopic: 77.029919919
Chemical FormulaC2H7NS
InChI KeyUFULAYFCSOUIOV-UHFFFAOYSA-N
InChI
InChI=1S/C2H7NS/c3-1-2-4/h4H,1-3H2
IUPAC Name
2-aminoethane-1-thiol
SMILES
NCCS
Mass Specshow(7.29 KB)
Taxonomy
KingdomOrganic Compounds
SuperclassOrganonitrogen Compounds
ClassAmines
SubclassPolyamines
Direct parentPolyamines
Alternative parentsAlkylthiols; Monoalkylamines
Substituentsprimary aliphatic amine; primary amine
Classification descriptionThis compound belongs to the polyamines. These are compounds containing more than one amine group.
Pharmacology
IndicationGiven intravenously or orally to treat radiation sickness. The bitartrate salts (Cystagon® and Procysbi) have been used for the oral treatment of nephropathic cystinosis and cystinurea. The hydrochloride salt (Cystaran™) is indicated for the treatment of corneal cystine crystal accumulation in cystinosis patients.
PharmacodynamicsPeople born without the ability to metabolize the amino acid cystine suffer from cystinosis, a rare inherited disorder characterized by the deposition and accumulation of cystine crystals throughout the body. These crystals cause considerable damage, particularly in the kidney and eye. Kidney failure can occur by the age of 10 in untreated patients. Cysteamine prevents the accumulation of cystine crystals and is prescribed to prevent further kidney and eye damage. Cysteamine helps to convert cystine into less harmful chemical forms that can be removed from cells.
Mechanism of actionThe free thiol cysteamine depletes cystinotic leukocytes and other cells of cystine, whose accumulation is considered the cause of organ damage in cystinosis. Cysteamine cleaves the disulfide bond with cystine to produce molecules that can escape the metabolic defect in cystinosis and cystinuria.
AbsorptionCystagon® reaches its maximum plasma concentration in about 1.4 hours.
Volume of distribution

Cystagon® has a volume of distribution of 156 L.

Protein bindingCysteamine has a plasma protein binding of 52% and is mostly bound to albumin.
Metabolism

The metabolism of cysteamine has not yet been determined.

Route of eliminationNot Available
Half lifeNot Available
Clearance

The plasma clearance is about 1.2 L/min.

ToxicitySymptoms of overdose may include convulsions (seizures), increased thirst and unusual tiredness or weakness.
Affected organisms
  • Humans and other mammals
PathwaysNot Available
SNP Mediated EffectsNot Available
SNP Mediated Adverse Drug ReactionsNot Available
ADMET
Predicted ADMET features
Property Value Probability
Human Intestinal Absorption + 0.9378
Blood Brain Barrier + 0.7618
Caco-2 permeable + 0.7461
P-glycoprotein substrate Non-substrate 0.7
P-glycoprotein inhibitor I Non-inhibitor 0.9634
P-glycoprotein inhibitor II Non-inhibitor 0.9637
Renal organic cation transporter Non-inhibitor 0.751
CYP450 2C9 substrate Non-substrate 0.9035
CYP450 2D6 substrate Non-substrate 0.5713
CYP450 3A4 substrate Non-substrate 0.8282
CYP450 1A2 substrate Non-inhibitor 0.9046
CYP450 2C9 substrate Non-inhibitor 0.9071
CYP450 2D6 substrate Non-inhibitor 0.9231
CYP450 2C19 substrate Non-inhibitor 0.9025
CYP450 3A4 substrate Non-inhibitor 0.9396
CYP450 inhibitory promiscuity Low CYP Inhibitory Promiscuity 0.8091
Ames test Non AMES toxic 0.8488
Carcinogenicity Non-carcinogens 0.5197
Biodegradation Not ready biodegradable 0.7564
Rat acute toxicity 2.2165 LD50, mol/kg Not applicable
hERG inhibition (predictor I) Weak inhibitor 0.8358
hERG inhibition (predictor II) Non-inhibitor 0.8686
Pharmacoeconomics
Manufacturers
  • Mylan pharmaceuticals inc
Packagers
Dosage forms
FormRouteStrength
CapsuleOralEQ 150MG BASE (CYSTAGON)
CapsuleOralEQ 50MG BASE (CYSTAGON )
Capsule, delayed releaseOral 25MG BASE (PROCYSBI)
Capsule, delayed releaseOral75MG BASE (PROCYSBI)
Solution / dropsOphthalmicEQ 0.44% BASE (CYSTARAN)
Prices
Unit descriptionCostUnit
Cystagon 150 mg capsule1.28USDcapsule
Cystagon 50 mg capsule0.44USDcapsule
DrugBank does not sell nor buy drugs. Pricing information is supplied for informational purposes only.
PatentsNot Available
Properties
Statesolid
Experimental Properties
PropertyValueSource
melting point98 °CPhysProp
water solubilityFreely soluble in water.From The Merck Index.
logP0.1Not Available
Predicted Properties
PropertyValueSource
Water Solubility23.5ALOGPS
logP0.01ALOGPS
logP-0.42ChemAxon
logS-0.52ALOGPS
pKa (Strongest Acidic)9.42ChemAxon
pKa (Strongest Basic)10.4ChemAxon
Physiological Charge1ChemAxon
Hydrogen Acceptor Count1ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area26.02 Å2ChemAxon
Rotatable Bond Count1ChemAxon
Refractivity22.39 m3·mol-1ChemAxon
Polarizability8.65 Å3ChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleNoChemAxon
Spectra
SpectraGC-MSMS/MS1D NMR2D NMR
References
Synthesis Reference

Tethuharu Okazaki, Takeo Komukai, Saburo Uchikuga, “Process for preparing cysteamine-S-substituted compounds and derivatives thereof.” U.S. Patent US4371472, issued September, 1969.

US4371472
General Reference
  1. Lukashin BP, Grebeniuk AN: [Comparative study of the radiation-protective effectiveness of low doses of cysteamine, heparin, and naphtizine in experiments on mice] Radiats Biol Radioecol. 2001 May-Jun;41(3):310-2. Pubmed
  2. Dohil R, Fidler M, Gangoiti JA, Kaskel F, Schneider JA, Barshop BA: Twice-daily cysteamine bitartrate therapy for children with cystinosis. J Pediatr. 2010 Jan;156(1):71-75.e1-3. doi: 10.1016/j.jpeds.2009.07.016. Epub . Pubmed
  3. FDA label.
External Links
ResourceLink
KEGG DrugD03634
KEGG CompoundC01678
PubChem Compound6058
PubChem Substance46507730
ChemSpider5834
BindingDB7968
ChEBI17141
ChEMBLCHEMBL602
Therapeutic Targets DatabaseDAP001297
PharmGKBPA449171
RxListhttp://www.rxlist.com/cystagon-drug.htm
Drugs.comhttp://www.drugs.com/cdi/cysteamine.html
WikipediaCysteamine
ATC CodesA16AA04
AHFS CodesNot Available
PDB EntriesNot Available
FDA labelshow(115 KB)
MSDSshow(73.7 KB)
Interactions
Drug InteractionsSearched, but no interactions found.
Food InteractionsNot Available

Targets

1. Cystine

Kind: amino acid

Organism: Human

Pharmacological action: unknown

Actions: cleavage

Components

Name UniProt ID Details

References:

  1. Omran Z, Kay G, Di Salvo A, Knott RM, Cairns D: PEGylated derivatives of cystamine as enhanced treatments for nephropathic cystinosis. Bioorg Med Chem Lett. 2011 Jan 1;21(1):45-7. Epub 2010 Nov 21. Pubmed

2. Somatostatin

Kind: protein

Organism: Human

Pharmacological action: unknown

Actions: binder

Components

Name UniProt ID Details
Somatostatin P61278 Details

References:

  1. Ahren B, Bottcher G, Ekman R, Sundler F: Cysteamine and the endocrine pancreas: immunocytochemical, immunochemical, and functional aspects. Cell Tissue Res. 1989 Apr;256(1):159-66. Pubmed
  2. McIntosh CH, Bakich V, Bokenfohr K, DiScala-Guenot D, Kwok YN, Brown JC: Cysteamine-induced reduction in gastrointestinal somatostatin: evidence for a region-specific loss in immunoreactivity. Regul Pept. 1988 Jun;21(3-4):205-18. Pubmed
  3. Terry LC, Craig R: Cysteamine effects on monoamines, dopamine-beta-hydroxylase and the hypothalamic-pituitary axis. Neuroendocrinology. 1985 Dec;41(6):467-75. Pubmed
  4. McIntosh C, Bakich V, Trotter T, Kwok YN, Nishimura E, Pederson R, Brown J: Effect of cysteamine on secretion of gastrin and somatostatin from the rat stomach. Gastroenterology. 1984 May;86(5 Pt 1):834-8. Pubmed
  5. Chen X, Ji ZL, Chen YZ: TTD: Therapeutic Target Database. Nucleic Acids Res. 2002 Jan 1;30(1):412-5. Pubmed

3. Neuropeptide Y receptor type 2

Kind: protein

Organism: Human

Pharmacological action: unknown

Actions: other/unknown

Components

Name UniProt ID Details
Neuropeptide Y receptor type 2 P49146 Details

References:

  1. Li W, Hexum TD: Cysteamine selectively enhances neuropeptide Y2 receptor binding activity. Biochem Biophys Res Commun. 1992 Apr 15;184(1):380-6. Pubmed

Enzymes

1. Myeloperoxidase

Kind: protein

Organism: Human

Pharmacological action: unknown

Actions: substrate

Components

Name UniProt ID Details
Myeloperoxidase P05164 Details

References:

  1. Svensson BE: Abilities of peroxidases to catalyse peroxidase-oxidase oxidation of thiols. Biochem J. 1988 Dec 15;256(3):757-62. Pubmed
  2. Svensson BE, Graslund A, Strom G, Moldeus P: Thiols as peroxidase substrates. Free Radic Biol Med. 1993 Feb;14(2):167-75. Pubmed
  3. Svensson BE, Lindvall S: Myeloperoxidase-oxidase oxidation of cysteamine. Biochem J. 1988 Jan 15;249(2):521-30. Pubmed

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Drug created on June 13, 2005 07:24 / Updated on September 16, 2013 17:12