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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
2-Amino-1-ethanethiol
2-AMINO-ethanethiol
2-Aminoethanethiol
beta-Aminoethanethiol
beta-Aminoethylthiol
beta-MEA
beta-Mercaptoethylamine
Cysteamine
MEA
Mercaptamina
Mercaptamine
Mercaptaminum
Thioethanolamine
β-aminoethylthiol
β-MEA
External Identifiers Not Available
Approved Prescription Products
NameDosageStrengthRouteLabellerMarketing StartMarketing End
Cystagoncapsule50 mg/1oralMylan Pharmaceuticals Inc.1994-08-15Not applicableUs
Cystagoncapsule150 mg/1oralMylan Pharmaceuticals Inc.1994-08-15Not applicableUs
Cystaransolution6.5 mg/mLophthalmicSigma Tau Pharmaceuticals, Inc.2012-12-30Not applicableUs
Procysbi Delayed-releasecapsule, delayed release pellets75 mg/1oralRaptor Therapeutics Inc.2013-04-30Not applicableUs
Procysbi Delayed-releasecapsule, delayed release pellets25 mg/1oralRaptor Therapeutics Inc.2013-04-30Not applicableUs
Approved Generic Prescription ProductsNot Available
Approved Over the Counter ProductsNot Available
Unapproved/Other Products Not 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
UNII5UX2SD1KE2
CAS number60-23-1
WeightAverage: 77.149
Monoisotopic: 77.029919919
Chemical FormulaC2H7NS
InChI KeyInChIKey=UFULAYFCSOUIOV-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.
Related Articles
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
Pathways
PathwayCategorySMPDB ID
Pantothenate and CoA BiosynthesisMetabolicSMP00027
Taurine and Hypotaurine MetabolismMetabolicSMP00021
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 inhibitorNon-inhibitor0.9071
CYP450 2D6 inhibitorNon-inhibitor0.9231
CYP450 2C19 inhibitorNon-inhibitor0.9025
CYP450 3A4 inhibitorNon-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
ADMET data is predicted using admetSAR, a free tool for evaluating chemical ADMET properties. (23092397 )
Pharmacoeconomics
Manufacturers
  • Mylan pharmaceuticals inc
Packagers
Dosage forms
FormRouteStrength
Capsuleoral150 mg/1
Capsuleoral50 mg/1
Solutionophthalmic6.5 mg/mL
Capsule, delayed release pelletsoral25 mg/1
Capsule, delayed release pelletsoral75 mg/1
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.
Patents
Patent NumberPediatric ExtensionApprovedExpires (estimated)
US8026284 No2007-09-222027-09-22Us
US9173851 No2014-06-172034-06-17Us
US9192590 No2007-01-262027-01-26Us
US9198882 No2007-01-262027-01-26Us
US9233077 No2014-06-172034-06-17Us
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)
Spectra
Spectrum TypeDescriptionSplash Key
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (3 TMS)splash10-00di-1900000000-287334efed4c27d47e62View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (3 TMS)splash10-00di-1900000000-e26f666cab18d523e475View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (3 TMS)splash10-00di-7900000000-25e27a3413a3e9bd6e86View in MoNA
GC-MSGC-MS Spectrum - GC-MS (3 TMS)splash10-00dr-3900000000-5f3c0dc6b99382c852e2View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-03di-9000000000-0fdf5bdcf8a4a1bf9afbView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-03di-9000000000-f99f2f10c6728595d6eaView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-01t9-9000000000-fa7f96e00debe1bebf70View in MoNA
1D NMR1H NMR SpectrumNot Available
2D NMR[1H,13C] 2D NMR SpectrumNot Available
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 References
  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:11458646 ]
  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:19775699 ]
External Links
ATC CodesA16AA04S01XA21
AHFS CodesNot Available
PDB EntriesNot Available
FDA labelDownload (115 KB)
MSDSDownload (73.7 KB)
Interactions
Drug Interactions
Drug
Aluminum hydroxideThe therapeutic efficacy of Cysteamine can be decreased when used in combination with Aluminum hydroxide.
Calcium carbonateThe therapeutic efficacy of Cysteamine can be decreased when used in combination with Calcium carbonate.
CimetidineThe therapeutic efficacy of Cysteamine can be decreased when used in combination with Cimetidine.
EsomeprazoleThe therapeutic efficacy of Cysteamine can be decreased when used in combination with Esomeprazole.
EthanolThe risk or severity of adverse effects can be increased when Ethanol is combined with Cysteamine.
FamotidineThe therapeutic efficacy of Cysteamine can be decreased when used in combination with Famotidine.
LansoprazoleThe therapeutic efficacy of Cysteamine can be decreased when used in combination with Lansoprazole.
Magnesium hydroxideThe therapeutic efficacy of Cysteamine can be decreased when used in combination with Magnesium hydroxide.
Magnesium oxideThe therapeutic efficacy of Cysteamine can be decreased when used in combination with Magnesium oxide.
NizatidineThe therapeutic efficacy of Cysteamine can be decreased when used in combination with Nizatidine.
OmeprazoleThe therapeutic efficacy of Cysteamine can be decreased when used in combination with Omeprazole.
PantoprazoleThe therapeutic efficacy of Cysteamine can be decreased when used in combination with Pantoprazole.
RabeprazoleThe therapeutic efficacy of Cysteamine can be decreased when used in combination with Rabeprazole.
RanitidineThe therapeutic efficacy of Cysteamine can be decreased when used in combination with Ranitidine.
Sodium bicarbonateThe therapeutic efficacy of Cysteamine can be decreased when used in combination with Sodium bicarbonate.
Food Interactions
  • Avoid administration of delayed release cysteamine less than 30 minutes before and within 2 hours after ingesting food due to decreased bioavailability of cysteamine.

Targets

1. Cystine
Kind
Amino acid
Organism
Human
Pharmacological action
unknown
Actions
cleavage
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. doi: 10.1016/j.bmcl.2010.11.085. Epub 2010 Nov 21. [PubMed:21147534 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
binder
General Function:
Hormone activity
Specific Function:
Somatostatin inhibits the release of somatotropin.
Gene Name:
SST
Uniprot ID:
P61278
Molecular Weight:
12735.395 Da
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:2653642 ]
  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:2901134 ]
  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:4080089 ]
  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:6142843 ]
  5. Chen X, Ji ZL, Chen YZ: TTD: Therapeutic Target Database. Nucleic Acids Res. 2002 Jan 1;30(1):412-5. [PubMed:11752352 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
other/unknown
General Function:
Receptor activity
Specific Function:
Receptor for neuropeptide Y and peptide YY. The rank order of affinity of this receptor for pancreatic polypeptides is PYY > NPY > PYY (3-36) > NPY (2-36) > [Ile-31, Gln-34] PP > [Leu-31, Pro-34] NPY > PP, [Pro-34] PYY and NPY free acid.
Gene Name:
NPY2R
Uniprot ID:
P49146
Molecular Weight:
42730.69 Da
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:1314592 ]

Enzymes

Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
substrate
General Function:
Peroxidase activity
Specific Function:
Part of the host defense system of polymorphonuclear leukocytes. It is responsible for microbicidal activity against a wide range of organisms. In the stimulated PMN, MPO catalyzes the production of hypohalous acids, primarily hypochlorous acid in physiologic situations, and other toxic intermediates that greatly enhance PMN microbicidal activity.
Gene Name:
MPO
Uniprot ID:
P05164
Molecular Weight:
83867.71 Da
References
  1. Svensson BE: Abilities of peroxidases to catalyse peroxidase-oxidase oxidation of thiols. Biochem J. 1988 Dec 15;256(3):757-62. [PubMed:2852004 ]
  2. Svensson BE, Graslund A, Strom G, Moldeus P: Thiols as peroxidase substrates. Free Radic Biol Med. 1993 Feb;14(2):167-75. [PubMed:8381104 ]
  3. Svensson BE, Lindvall S: Myeloperoxidase-oxidase oxidation of cysteamine. Biochem J. 1988 Jan 15;249(2):521-30. [PubMed:2829860 ]
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Drug created on June 13, 2005 07:24 / Updated on May 28, 2016 02:12