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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
Prescription Products
NameDosageStrengthRouteLabellerMarketing StartMarketing End
Cystagoncapsule50 mgoralMylan Pharmaceuticals Inc.1994-08-15Not AvailableUs 0a2ef1ad1c84951dc1392a8bbe1f3cb241c91ed59e44ad8268635315440d978c
Cystagoncapsule150 mgoralMylan Pharmaceuticals Inc.1994-08-15Not AvailableUs 0a2ef1ad1c84951dc1392a8bbe1f3cb241c91ed59e44ad8268635315440d978c
Cystaransolution6.5 mg/mLophthalmicSigma Tau Pharmaceuticals, Inc.2012-12-30Not AvailableUs 0a2ef1ad1c84951dc1392a8bbe1f3cb241c91ed59e44ad8268635315440d978c
Generic Prescription ProductsNot Available
Over the Counter ProductsNot Available
International Brands
NameCompany
ProcysbiRaptor Pharms
Brand mixturesNot Available
Salts
Name/CASStructureProperties
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
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
Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as alkylthiols. These are organic compounds containing the thiol functional group linked to an alkyl chain.
KingdomOrganic compounds
Super ClassOrganosulfur compounds
ClassThiols
Sub ClassAlkylthiols
Direct ParentAlkylthiols
Alternative Parents
Substituents
  • Alkylthiol
  • Hydrocarbon derivative
  • Primary amine
  • Organonitrogen compound
  • Primary aliphatic amine
  • Amine
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
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
PropertyValueProbability
Human Intestinal Absorption+0.9378
Blood Brain Barrier+0.7618
Caco-2 permeable+0.7461
P-glycoprotein substrateNon-substrate0.7
P-glycoprotein inhibitor INon-inhibitor0.9634
P-glycoprotein inhibitor IINon-inhibitor0.9637
Renal organic cation transporterNon-inhibitor0.751
CYP450 2C9 substrateNon-substrate0.9035
CYP450 2D6 substrateNon-substrate0.5713
CYP450 3A4 substrateNon-substrate0.8282
CYP450 1A2 substrateNon-inhibitor0.9046
CYP450 2C9 substrateNon-inhibitor0.9071
CYP450 2D6 substrateNon-inhibitor0.9231
CYP450 2C19 substrateNon-inhibitor0.9025
CYP450 3A4 substrateNon-inhibitor0.9396
CYP450 inhibitory promiscuityLow CYP Inhibitory Promiscuity0.8091
Ames testNon AMES toxic0.8488
CarcinogenicityNon-carcinogens0.5197
BiodegradationNot ready biodegradable0.7564
Rat acute toxicity2.2165 LD50, mol/kg Not applicable
hERG inhibition (predictor I)Weak inhibitor0.8358
hERG inhibition (predictor II)Non-inhibitor0.8686
Pharmacoeconomics
Manufacturers
  • Mylan pharmaceuticals inc
Packagers
Dosage forms
FormRouteStrength
Capsuleoral150 mg
Capsuleoral50 mg
Solutionophthalmic6.5 mg/mL
Prices
Unit descriptionCostUnit
Cystagon 150 mg capsule1.28USD capsule
Cystagon 50 mg capsule0.44USD capsule
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 Solubility23.5 mg/mLALOGPS
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 FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Mass Spec (NIST)Download (7.29 KB)
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
ATC CodesA16AA04S01XA21
AHFS CodesNot Available
PDB EntriesNot Available
FDA labelDownload (115 KB)
MSDSDownload (73.7 KB)
Interactions
Drug InteractionsNo 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