CYSTEAMINE HYDROCHLORIDE

Details

Stereochemistry	ACHIRAL
Molecular Formula	C2H7NS.ClH
Molecular Weight	113.61
Optical Activity	NONE
Defined Stereocenters	0 / 0
E/Z Centers	0
Charge	0

SHOW SMILES / InChI

Structure Search

SMILES

Cl.NCCS

InChI

InChIKey=OGMADIBCHLQMIP-UHFFFAOYSA-N

InChI=1S/C2H7NS.ClH/c3-1-2-4;/h4H,1-3H2;1H

HIDE SMILES / InChI

Description
Sources: https://www.accessdata.fda.gov/drugsatfda_docs/label/2007/020392s010lbl.pdf
Curator's Comment: description was created based on several sources, including https://www.ncbi.nlm.nih.gov/pubmed/6131501 | https://www.ncbi.nlm.nih.gov/pubmed/6124307 | https://www.ncbi.nlm.nih.gov/pubmed/15675041 | https://www.ncbi.nlm.nih.gov/pubmed/28384851

Cysteamine (trade name CYSTAGON) is a cystine-depleting agent indicated for the treatment of corneal cystine crystal accumulation in patients with cystinosis. Cystinosis is an autosomal recessive inborn error of metabolism in which the transport of cystine out of lysosomes is abnormal; in the nephropathic form, accumulation of cystine and formation of crystals damage various organs, especially the kidney, leading to renal tubular Fanconi Syndrome and progressive glomerular failure, with end-stage renal failure by the end of the first decade of life. In four studies of cystinosis patients before cysteamine was available, renal death (need for transplant or dialysis) occurred at the median age of fewer than 10 years. Patients with cystinosis also experience growth failure, rickets, and photophobia due to cystine deposits in the cornea. With time most organs are damaged, including the retina, muscles and central nervous system. Cysteamine is an aminothiol that participates within lysosomes in a thiol-disulfide interchange reaction converting cystine into cysteine and cysteine-cysteamine mixed disulfide, both of which can exit the lysosome in patients with cystinosis.

CNS Activity

Originator

Approval Year

Targets

Primary Target	Pharmacology	Condition	Potency
Protein-glutamine gamma-glutamyltransferase 2 7 Target ID: P21980 Gene ID: 7052.0 Gene Symbol: TGM2 Target Organism: Homo sapiens (Human) Sources: https://www.ncbi.nlm.nih.gov/pubmed/15675041	Inhibitor 8297		178.0 µM [IC50]

Conditions

Condition	Modality	Targets	Highest Phase	Product
Nephropathic cystinosis 6 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/label/2007/020392s010lbl.pdf	Primary		Approved 8429	CYSTAGON Approved Use PROCYSBI is indicated for the treatment of nephropathic cystinosis in adult and pediatric patients 2 years of age and older. PROCYSBI is a cystine-depleting agent indicated for the treatment of nephropathic cystinosis in adult and pediatric patients 2 years of age and older. (1) Launch Date 1994

C_max

Value	Dose	Co-administered	Analyte	Population
2.7 mg/L DRUG LABEL https://www.accessdata.fda.gov/drugsatfda_docs/label/2020/213491s000lbl.pdf	404 mg/m² 4 times / day steady-state, oral dose: 404 mg/m² route of administration: Oral experiment type: STEADY-STATE co-administered:		CYSTEAMINE plasma	Homo sapiens population: UNHEALTHY age: CHILD sex: FEMALE / MALE food status: UNKNOWN
37.72 μM DRUG LABEL https://www.accessdata.fda.gov/drugsatfda_docs/label/2013/203389s000lbl.pdf	450 mg single, oral dose: 450 mg route of administration: Oral experiment type: SINGLE co-administered:		CYSTEAMINE plasma	Homo sapiens population: UNHEALTHY age: CHILD sex: FEMALE / MALE food status: UNKNOWN

AUC

Value	Dose	Co-administered	Analyte	Population
351 mg × min/L DRUG LABEL https://www.accessdata.fda.gov/drugsatfda_docs/label/2020/213491s000lbl.pdf	404 mg/m² 4 times / day steady-state, oral dose: 404 mg/m² route of administration: Oral experiment type: STEADY-STATE co-administered:		CYSTEAMINE plasma	Homo sapiens population: UNHEALTHY age: CHILD sex: FEMALE / MALE food status: UNKNOWN
96 μM × h DRUG LABEL https://www.accessdata.fda.gov/drugsatfda_docs/label/2013/203389s000lbl.pdf	450 mg single, oral dose: 450 mg route of administration: Oral experiment type: SINGLE co-administered:		CYSTEAMINE plasma	Homo sapiens population: UNHEALTHY age: CHILD sex: FEMALE / MALE food status: UNKNOWN

T_1/2

Value	Dose	Co-administered	Analyte	Population
90 min DRUG LABEL https://www.accessdata.fda.gov/drugsatfda_docs/label/2020/213491s000lbl.pdf	404 mg/m² 4 times / day steady-state, oral dose: 404 mg/m² route of administration: Oral experiment type: STEADY-STATE co-administered:		CYSTEAMINE plasma	Homo sapiens population: UNHEALTHY age: CHILD sex: FEMALE / MALE food status: UNKNOWN

F_unbound

Value	Dose	Co-administered	Analyte	Population
48% DRUG LABEL https://www.accessdata.fda.gov/drugsatfda_docs/label/2020/213491s000lbl.pdf	404 mg/m² 4 times / day steady-state, oral dose: 404 mg/m² route of administration: Oral experiment type: STEADY-STATE co-administered:		CYSTEAMINE plasma	Homo sapiens population: UNHEALTHY age: CHILD sex: FEMALE / MALE food status: UNKNOWN
48% DRUG LABEL https://www.accessdata.fda.gov/drugsatfda_docs/label/2013/203389s000lbl.pdf	450 mg single, oral dose: 450 mg route of administration: Oral experiment type: SINGLE co-administered:		CYSTEAMINE plasma	Homo sapiens population: UNHEALTHY age: CHILD sex: FEMALE / MALE food status: UNKNOWN

Doses

Dose	Population	Adverse events
0.44 % 1 times / day multiple, ophthalmic Recommended Dose: 0.44 %, 1 times / day Route: ophthalmic Route: multiple Dose: 0.44 %, 1 times / day Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2012/200740Orig1s000MedR.pdf Page: 98 EI0109S	unhealthy, 13.4 (5.9-27.8) n = 20 Health Status: unhealthy Condition: cystinosis Age Group: 13.4 (5.9-27.8) Sex: M+F Population Size: 20 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2012/200740Orig1s000MedR.pdf Page: 98 EI0109S	Disc. AE: Intracranial hypertension... AEs leading to discontinuation/dose reduction: Intracranial hypertension Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2012/200740Orig1s000MedR.pdf Page: 98 EI0109S
600 mg 1 times / day multiple, oral Recommended Dose: 600 mg, 1 times / day Route: oral Route: multiple Dose: 600 mg, 1 times / day Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000MedR.pdf Page: RP103-05	healthy, 37.5 (19-64) n = 20 Health Status: healthy Age Group: 37.5 (19-64) Sex: M+F Population Size: 20 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000MedR.pdf Page: RP103-05	Disc. AE: Blurred vision, Hematuria... AEs leading to discontinuation/dose reduction: Blurred vision Hematuria Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000MedR.pdf Page: RP103-05

AEs

AE	Significance	Dose	Population
Intracranial hypertension	Disc. AE	0.44 % 1 times / day multiple, ophthalmic Recommended Dose: 0.44 %, 1 times / day Route: ophthalmic Route: multiple Dose: 0.44 %, 1 times / day Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2012/200740Orig1s000MedR.pdf Page: 98 EI0109S	unhealthy, 13.4 (5.9-27.8) n = 20 Health Status: unhealthy Condition: cystinosis Age Group: 13.4 (5.9-27.8) Sex: M+F Population Size: 20 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2012/200740Orig1s000MedR.pdf Page: 98 EI0109S
Blurred vision	Disc. AE	600 mg 1 times / day multiple, oral Recommended Dose: 600 mg, 1 times / day Route: oral Route: multiple Dose: 600 mg, 1 times / day Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000MedR.pdf Page: RP103-05	healthy, 37.5 (19-64) n = 20 Health Status: healthy Age Group: 37.5 (19-64) Sex: M+F Population Size: 20 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000MedR.pdf Page: RP103-05
Hematuria	Disc. AE	600 mg 1 times / day multiple, oral Recommended Dose: 600 mg, 1 times / day Route: oral Route: multiple Dose: 600 mg, 1 times / day Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000MedR.pdf Page: RP103-05	healthy, 37.5 (19-64) n = 20 Health Status: healthy Age Group: 37.5 (19-64) Sex: M+F Population Size: 20 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000MedR.pdf Page: RP103-05

Overview

CYP3A4	CYP2C9	CYP2D6	hERG
substrate 1737 inhibitor 1587 inducer 781	substrate 1037 inhibitor 1767	substrate 1217 inhibitor 1852

OverviewOther

Other Inhibitor	Other Substrate	Other Inducer
CYP1A2 1524 CYP2B6 810 CYP2C8 911 CYP2C19 1478 CYP2E1 882 CYP2A6 707 P-gp 1461 BCRP 699 OATP1B1 788 OATP1B3 706 OAT1 599 OAT3 642 OCT1 512 OCT2 647	CYP1A2 1054 CYP2B6 664 CYP2C8 693 CYP2C19 991 CYP2E1 667 CYP2A6 543 P-gp 1537 BCRP 561 OATP1B1 406 OATP1B3 350 OAT1 326 OAT3 339 OCT1 327 OCT2 355	CYP1A2 701 CYP2B6 547

Drug as perpetrator

Target	Modality	Activity
CYP1A2 1692	inducer 1573 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=22 Page: 22.0	inconclusive
CYP2B6 1001	inducer 1573 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=22 Page: 22.0	inconclusive
CYP3A4 1925	inducer 1573 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=22 Page: 22.0	inconclusive
BCRP 773	inhibitor 3277 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=22 Page: 22.0	no
CYP1A2 1692	inhibitor 3277 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=20 Page: 20.0	no
CYP2A6 739	inhibitor 3277 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=20 Page: 20.0	no
CYP2B6 1001	inhibitor 3277 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=20 Page: 20.0	no
CYP2C19 1553	inhibitor 3277 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=20 Page: 20.0	no
CYP2C8 965	inhibitor 3277 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=20 Page: 20.0	no
CYP2C9 1819	inhibitor 3277 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=20 Page: 20.0	no
CYP2D6 1891	inhibitor 3277 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=20 Page: 20.0	no
CYP2E1 970	inhibitor 3277 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=20 Page: 20.0	no
CYP3A4 1925	inhibitor 3277 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=20 Page: 20.0	no
OAT1 603	inhibitor 3277 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=22 Page: 22.0	no
OAT3 644	inhibitor 3277 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=22 Page: 22.0	no
OATP1B1 803	inhibitor 3277 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=22 Page: 22.0	no
OATP1B3 715	inhibitor 3277 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=22 Page: 22.0	no
P-gp 1650	inhibitor 3277 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=22 Page: 22.0	no
OCT1 543	inhibitor 3277 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=22 Page: 22.0	weak [Inhibition 780 uM]
OCT2 648	inhibitor 3277 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=22 Page: 22.0	weak [Inhibition 780 uM]

Drug as victim

Target	Modality	Activity
BCRP 561	substrate 3367 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=22 Page: 22.0	no
CYP2A6 543	substrate 3367 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=16 Page: 16.0	no
CYP3A4 1737	substrate 3367 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=16 Page: 16.0	no
OAT1 326	substrate 3367 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=23 Page: 23.0	no
OAT3 339	substrate 3367 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=23 Page: 23.0	no
OATP1B1 406	substrate 3367 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=23 Page: 23.0	no
OATP1B3 350	substrate 3367 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=23 Page: 23.0	no
OCT1 327	substrate 3367 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=23 Page: 23.0	no
CYP1A2 1054	substrate 3367 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=16 Page: 16.0	yes
CYP2B6 664	substrate 3367 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=16 Page: 16.0	yes
CYP2C19 991	substrate 3367 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=16 Page: 16.0	yes
CYP2C8 693	substrate 3367 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=16 Page: 16.0	yes
CYP2C9 1037	substrate 3367 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=16 Page: 16.0	yes
CYP2D6 1217	substrate 3367 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=16 Page: 16.0	yes
CYP2E1 667	substrate 3367 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=16 Page: 16.0	yes
OCT2 355	substrate 3367 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=23 Page: 23.0	yes
P-gp 1537	substrate 3367 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=22 Page: 22.0	yes

PubMed

Title	Date	PubMed
Taurine and hypotaurine in spermatozoa and epididymal fluid of cats.	2001	11787195
Oxidative stress, glucose-6-phosphate dehydrogenase and the red cell.	2001	11765001
Effects of cysteamine on nociception in mice.	2001 Jul	11749823
Effect of centrally administered oxytocin on gastric and duodenal ulcers in rats.	2001 Jun	11747752
Adsorption properties and activities of lipase on a gold substrate modified by self-assembled monolayers.	2001 Nov	11791710
Gastric antisecretory and antiulcer activity of oxytocin in rats and guinea pigs.	2001 Nov 21	11764003
Role of porphyrin sequestration in the biogenesis of iron-laden astrocytic inclusions in primary culture.	2002	12401955
Role of heme oxygenase-1 in the biogenesis of corpora amylacea.	2002	12232504
A novel strategy for the synthesis of neoglycoconjugates from deacylated deep rough lipopolysaccharides.	2002	12230919
In vitro maturation and glutathione synthesis of porcine oocytes in the presence or absence of cysteamine under different oxygen tensions: role of cumulus cells.	2002	12219933
Interventions for paracetamol (acetaminophen) overdoses.	2002	12137690
Enhancement of solar inactivation of Escherichia coli by titanium dioxide photocatalytic oxidation.	2002	11972697
Electrochemical and spectroelectrochemical behavior of the main photodegradation product of nifedipine: the nitrosopyridine derivative.	2002 Apr	12033390
Modulation of inflammatory paw oedema by cysteamine in the rat.	2002 Apr	12030790
Effects of tea from Turnera ulmifolia L. on mouse gastric mucosa support the Turneraceae as a new source of antiulcerogenic drugs.	2002 Apr	11995930
Generation of roasted notes based on 2-acetyl-2-thiazoline and its precursor, 2-(1-hydroxyethyl)-4,5-dihydrothiazole, by combined bio and thermal approaches.	2002 Apr 10	11929296
Effect of sperm survival and CTC staining pattern on in vitro fertilization of porcine oocytes.	2002 Apr 15	12041695
The ability of hippocampal CA1 area for induction of long-term potentiation is persistently reduced by prior treatment with cysteamine: an in vitro study.	2002 Aug	12372700
Differentiating the orientations of photosynthetic reaction centers on Au electrodes linked by different bifunctional reagents.	2002 Aug	12052357
In vivo confocal microscopy of the cornea in nephropathic cystinosis.	2002 Dec	12470153
Glutathione in gingival crevicular fluid and its relation to local antioxidant capacity in periodontal health and disease.	2002 Dec	12456773
Lysosomal cystine storage augments apoptosis in cultured human fibroblasts and renal tubular epithelial cells.	2002 Dec	12444206
Evidence for a central inhibitory role of growth hormone secretagogues and ghrelin on gastric acid secretion in conscious rats.	2002 Feb	11867937
Generating favorable nano-environments for thermal and solvent stabilization of immobilized beta-galactosidase.	2002 Feb 15	11787015
A molecular CT blood pool contrast agent.	2002 Jul	12139092
Cysteine-mediated electron transfer in syntrophic acetate oxidation by cocultures of Geobacter sulfurreducens and Wolinella succinogenes.	2002 Jul	12070769
Cystinosis.	2002 Jul 11	12110740
Synthesis of (+/-)-madindolines and chemical models. Studies of chemical reactivity.	2002 Jul 11	12098241
Gastroprotective effect of adrenomedullin administered subcutaneously in the rat.	2002 Jun	12126744
X-ray crystallographic studies on butyryl-ACP reveal flexibility of the structure around a putative acyl chain binding site.	2002 Jun	12057197
Cysteamine supplementation during in vitro maturation and embryo culture: a useful tool for increasing the efficiency of bovine in vitro embryo production.	2002 Jun	11984830
The effects of lysosomotropic agents on normal and INCL cells provide further evidence for the lysosomal nature of palmitoyl-protein thioesterase function.	2002 Jun 13	12069847
Age-related prevalence of anterior segment complications in patients with infantile nephropathic cystinosis.	2002 Mar	11862089
Prevalence of apoptosis and inner cell allocation in bovine embryos cultured under different oxygen tensions with or without cysteine addition.	2002 Mar 15	12054204
Effect of glutathione synthesis stimulation during in vitro maturation of ovine oocytes on embryo development and intracellular peroxide content.	2002 Mar 15	12054203
Polymer-cysteamine conjugates: new mucoadhesive excipients for drug delivery?	2002 Mar 2	11839440
Subunit modification and association in VR1 ion channels.	2002 Mar 22	11914134
Negligible urinary cysteamine loss in cystinosis patients with Fanconi syndrome.	2002 May	12036193
Properties of selected S-nitrosothiols compared to nitrosylated WR-1065.	2002 May	11966324
Endogenous somatostatin inhibits interaction of insulin and cholecystokinin on exocrine secretion of isolated, perfused rat pancreas.	2002 May	11961490
Tumour targeting of humanised cross-linked divalent-Fab' antibody fragments: a clinical phase I/II study.	2002 May 6	11986771
Characterization of the surfactin synthetase C-terminal thioesterase domain as a cyclic depsipeptide synthase.	2002 Nov 12	12416979
Cysteamine pre-treatment reduces pentylenetetrazol-induced plasticity and epileptiform discharge in the CA1 region of rat hippocampal slices.	2002 Nov 15	12419525
Nephropathic cystinosis associated with cardiomyopathy: a 27-year clinical follow-up.	2002 Nov 9	12425721
Follow-up and treatment of adults with cystinosis in the Netherlands.	2002 Oct	12270982
Possible mechanism for involvement of cysteine in aroma production in wine.	2002 Oct 9	12358496
Studies on the anti-inflammatory and anti-nociceptive effects of melatonin in the rat.	2002 Sep	12220966
Identification and quantitation of key aroma compounds formed in Maillard-type reactions of fructose with cysteamine or isothiaproline (1,3-thiazolidine-2-carboxylic acid).	2002 Sep 11	12207481
Structural determination and odor characterization of N-(2-mercaptoethyl)-1,3-thiazolidine, a new intense popcorn-like-smelling odorant.	2002 Sep 11	12207480
Electric potential control of DNA immobilization on gold electrode.	2003 Jan	12445444

Patents

Sample Use Guides

In Vivo Use Guide

The recommended maintenance dose of 1.30 grams/m2/day. Patients over age 12 and over 110 pounds should receive 2.0 grams/day given in four divided doses as a starting maintenance dose. This dose should be reached after 4 to 6 weeks of incremental dosage increases as stated above. The dose should be raised if the leukocyte cystine level remains > 2 nmol/1⁄2 cystine/mg/protein.

Route of Administration: Oral

In Vitro Use Guide

Oocytes were obtained from 4-6 weeks old Naval Medical Research Institute (NMRI) female mice, 48 hours after stimulation with Intraperitoneal (IP) injection of 10 IU Pregnant Mare Serum Gonadotropin (PMSG). Germinal Vesicle (GV) oocyte with and without cumulus cells were isolated from ovaries and cultured in Tissue Culture Medium (TCM) 199 with availability of 100 μM of CYS (Cysteamine). After 24 hours, mature oocyte in metaphase II (MII) were fertilized with sperm in In vitro Fertilization (IVF) medium (T6) and evaluated for fetal development into blastocyst. CYS could significantly (p<0.05) increase the rate of IVM and oocyte evolution, and embryo formation in medium culture.

Name

Type

Language

CYSTEAMINE HYDROCHLORIDE

Official Name

English

CYSTEAMINE HYDROCHLORIDE [VANDF]

Common Name

English

NSC-758692

Code

English

CYSTEAMINE HCL

Official Name

English

CYSTADROPS

Brand Name

English

CYSTEAMINE HYDROCHLORIDE [USAN]

Common Name

English

NSC-21116

Code

English

NSC-25116

Code

English

CYSTEAMINE HYDROCHLORIDE [MI]

Common Name

English

CI-9148

Code

English

MERCAPTOETHYLAMINE HYDROCHLORIDE

Systematic Name

English

Mercaptamine hydrochloride [WHO-DD]

Common Name

English

MERCAPTAMINE HYDROCHLORIDE [MART.]

Common Name

English

MERCAPTAMINE HYDROCHLORIDE

Common Name

English

CYSTEAMINE HYDROCHLORIDE [ORANGE BOOK]

Common Name

English

ETHANETHIOL, 2-AMINO-, HYDROCHLORIDE

Systematic Name

English

2-Aminoethanethiol hydrochloride

Systematic Name

English

CYSTEAMINE HCL [INCI]

Common Name

English

Classification Tree Code System Code

FDA ORPHAN DRUG

105997