CYSTEAMINE BITARTRATE

Details

Stereochemistry	ABSOLUTE
Molecular Formula	C4H6O6.C2H7NS
Molecular Weight	227.236
Optical Activity	UNSPECIFIED
Defined Stereocenters	2 / 2
E/Z Centers	0
Charge	0

SHOW SMILES / InChI

Structure Search

SMILES

NCCS.O[C@H]([C@@H](O)C(O)=O)C(O)=O

InChI

InChIKey=NSKJTUFFDRENDM-ZVGUSBNCSA-N

InChI=1S/C4H6O6.C2H7NS/c5-1(3(7)8)2(6)4(9)10;3-1-2-4/h1-2,5-6H,(H,7,8)(H,9,10);4H,1-3H2/t1-,2-;/m1./s1

HIDE SMILES / InChI

Molecular Formula	C2H7NS
Molecular Weight	77.149
Charge	0
Count

Stereochemistry	ACHIRAL
Additional Stereochemistry	No
Defined Stereocenters	0 / 0
E/Z Centers	0
Optical Activity	NONE

Molecular Formula	C4H6O6
Molecular Weight	150.0868
Charge	0
Count

Stereochemistry	ABSOLUTE
Additional Stereochemistry	No
Defined Stereocenters	2 / 2
E/Z Centers	0
Optical Activity	UNSPECIFIED

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 8504		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 8583	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
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
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

AUC

Value	Dose	Co-administered	Analyte	Population
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
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

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/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
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

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	unhealthy, 13.4 (5.9-27.8) Health Status: unhealthy Age Group: 13.4 (5.9-27.8) Sex: M+F Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2012/200740Orig1s000MedR.pdf	Disc. AE: Intracranial hypertension... AEs leading to discontinuation/dose reduction: Intracranial hypertension Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2012/200740Orig1s000MedR.pdf
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	healthy, 37.5 (19-64) Health Status: healthy Age Group: 37.5 (19-64) Sex: M+F Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000MedR.pdf	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

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	unhealthy, 13.4 (5.9-27.8) Health Status: unhealthy Age Group: 13.4 (5.9-27.8) Sex: M+F Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2012/200740Orig1s000MedR.pdf
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	healthy, 37.5 (19-64) Health Status: healthy Age Group: 37.5 (19-64) Sex: M+F Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000MedR.pdf
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	healthy, 37.5 (19-64) Health Status: healthy Age Group: 37.5 (19-64) Sex: M+F Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000MedR.pdf

Overview

CYP3A4	CYP2C9	CYP2D6	hERG
substrate 1946 inhibitor 2252 inducer 1087	substrate 1193 inhibitor 2438	substrate 1388 inhibitor 2507

OverviewOther

Other Inhibitor	Other Substrate	Other Inducer
CYP1A2 2153 CYP2B6 926 CYP2C8 1057 CYP2C19 2057 CYP2E1 1060 CYP2A6 879 P-gp 1741 BCRP 910 OATP1B1 1079 OATP1B3 961 OAT1 725 OAT3 747 OCT1 620 OCT2 779	CYP1A2 1197 CYP2B6 776 CYP2C8 810 CYP2C19 1119 CYP2E1 729 CYP2A6 626 P-gp 2052 BCRP 697 OATP1B1 503 OATP1B3 435 OAT1 395 OAT3 391 OCT1 393 OCT2 434	CYP1A2 832 CYP2B6 669

Drug as perpetrator

Target	Modality	Activity
CYP1A2 2333	inducer 1966 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=22 Page: 22.0	inconclusive
CYP2B6 1160	inducer 1966 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=22 Page: 22.0	inconclusive
CYP3A4 2633	inducer 1966 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=22 Page: 22.0	inconclusive
BCRP 985	inhibitor 4027 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=22 Page: 22.0	no
CYP1A2 2333	inhibitor 4027 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=20 Page: 20.0	no
CYP2A6 911	inhibitor 4027 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=20 Page: 20.0	no
CYP2B6 1160	inhibitor 4027 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=20 Page: 20.0	no
CYP2C19 2141	inhibitor 4027 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=20 Page: 20.0	no
CYP2C8 1117	inhibitor 4027 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=20 Page: 20.0	no
CYP2C9 2497	inhibitor 4027 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=20 Page: 20.0	no
CYP2D6 2546	inhibitor 4027 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=20 Page: 20.0	no
CYP2E1 1146	inhibitor 4027 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=20 Page: 20.0	no
CYP3A4 2633	inhibitor 4027 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=20 Page: 20.0	no
OAT1 730	inhibitor 4027 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=22 Page: 22.0	no
OAT3 749	inhibitor 4027 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=22 Page: 22.0	no
OATP1B1 1095	inhibitor 4027 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=22 Page: 22.0	no
OATP1B3 970	inhibitor 4027 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=22 Page: 22.0	no
P-gp 1932	inhibitor 4027 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=22 Page: 22.0	no
OCT1 656	inhibitor 4027 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=22 Page: 22.0	weak [Inhibition 780 uM]
OCT2 782	inhibitor 4027 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 697	substrate 4014 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=22 Page: 22.0	no
CYP2A6 626	substrate 4014 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=16 Page: 16.0	no
CYP3A4 1946	substrate 4014 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=16 Page: 16.0	no
OAT1 395	substrate 4014 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=23 Page: 23.0	no
OAT3 391	substrate 4014 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=23 Page: 23.0	no
OATP1B1 503	substrate 4014 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=23 Page: 23.0	no
OATP1B3 435	substrate 4014 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=23 Page: 23.0	no
OCT1 393	substrate 4014 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=23 Page: 23.0	no
CYP1A2 1197	substrate 4014 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=16 Page: 16.0	yes
CYP2B6 776	substrate 4014 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=16 Page: 16.0	yes
CYP2C19 1119	substrate 4014 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=16 Page: 16.0	yes
CYP2C8 810	substrate 4014 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=16 Page: 16.0	yes
CYP2C9 1193	substrate 4014 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=16 Page: 16.0	yes
CYP2D6 1388	substrate 4014 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=16 Page: 16.0	yes
CYP2E1 729	substrate 4014 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=16 Page: 16.0	yes
OCT2 434	substrate 4014 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=23 Page: 23.0	yes
P-gp 2052	substrate 4014 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2013/203389Orig1s000ClinPharmR.pdf#page=22 Page: 22.0	yes

PubMed

Title	Date	PubMed
Electric potential control of DNA immobilization on gold electrode.	2003-01	12445444
In vivo confocal microscopy of the cornea in nephropathic cystinosis.	2002-12	12470153
Glutathione in gingival crevicular fluid and its relation to local antioxidant capacity in periodontal health and disease.	2002-12	12456773
Lysosomal cystine storage augments apoptosis in cultured human fibroblasts and renal tubular epithelial cells.	2002-12	12444206
Cysteamine pre-treatment reduces pentylenetetrazol-induced plasticity and epileptiform discharge in the CA1 region of rat hippocampal slices.	2002-11-15	12419525
Characterization of the surfactin synthetase C-terminal thioesterase domain as a cyclic depsipeptide synthase.	2002-11-12	12416979
Nephropathic cystinosis associated with cardiomyopathy: a 27-year clinical follow-up.	2002-11-09	12425721
Possible mechanism for involvement of cysteine in aroma production in wine.	2002-10-09	12358496
Follow-up and treatment of adults with cystinosis in the Netherlands.	2002-10	12270982
Identification and quantitation of key aroma compounds formed in Maillard-type reactions of fructose with cysteamine or isothiaproline (1,3-thiazolidine-2-carboxylic acid).	2002-09-11	12207481
Structural determination and odor characterization of N-(2-mercaptoethyl)-1,3-thiazolidine, a new intense popcorn-like-smelling odorant.	2002-09-11	12207480
Studies on the anti-inflammatory and anti-nociceptive effects of melatonin in the rat.	2002-09	12220966
The ability of hippocampal CA1 area for induction of long-term potentiation is persistently reduced by prior treatment with cysteamine: an in vitro study.	2002-08	12372700
Differentiating the orientations of photosynthetic reaction centers on Au electrodes linked by different bifunctional reagents.	2002-08	12052357
Cystinosis.	2002-07-11	12110740
Synthesis of (+/-)-madindolines and chemical models. Studies of chemical reactivity.	2002-07-11	12098241
A molecular CT blood pool contrast agent.	2002-07	12139092
Cysteine-mediated electron transfer in syntrophic acetate oxidation by cocultures of Geobacter sulfurreducens and Wolinella succinogenes.	2002-07	12070769
The effects of lysosomotropic agents on normal and INCL cells provide further evidence for the lysosomal nature of palmitoyl-protein thioesterase function.	2002-06-13	12069847
Gastroprotective effect of adrenomedullin administered subcutaneously in the rat.	2002-06	12126744
X-ray crystallographic studies on butyryl-ACP reveal flexibility of the structure around a putative acyl chain binding site.	2002-06	12057197
Cysteamine supplementation during in vitro maturation and embryo culture: a useful tool for increasing the efficiency of bovine in vitro embryo production.	2002-06	11984830
Tumour targeting of humanised cross-linked divalent-Fab' antibody fragments: a clinical phase I/II study.	2002-05-06	11986771
Negligible urinary cysteamine loss in cystinosis patients with Fanconi syndrome.	2002-05	12036193
Properties of selected S-nitrosothiols compared to nitrosylated WR-1065.	2002-05	11966324
Endogenous somatostatin inhibits interaction of insulin and cholecystokinin on exocrine secretion of isolated, perfused rat pancreas.	2002-05	11961490
Effect of sperm survival and CTC staining pattern on in vitro fertilization of porcine oocytes.	2002-04-15	12041695
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.	2002-04-10	11929296
Electrochemical and spectroelectrochemical behavior of the main photodegradation product of nifedipine: the nitrosopyridine derivative.	2002-04	12033390
Modulation of inflammatory paw oedema by cysteamine in the rat.	2002-04	12030790
Effects of tea from Turnera ulmifolia L. on mouse gastric mucosa support the Turneraceae as a new source of antiulcerogenic drugs.	2002-04	11995930
Subunit modification and association in VR1 ion channels.	2002-03-22	11914134
Prevalence of apoptosis and inner cell allocation in bovine embryos cultured under different oxygen tensions with or without cysteine addition.	2002-03-15	12054204
Effect of glutathione synthesis stimulation during in vitro maturation of ovine oocytes on embryo development and intracellular peroxide content.	2002-03-15	12054203
Polymer-cysteamine conjugates: new mucoadhesive excipients for drug delivery?	2002-03-02	11839440
Age-related prevalence of anterior segment complications in patients with infantile nephropathic cystinosis.	2002-03	11862089
Generating favorable nano-environments for thermal and solvent stabilization of immobilized beta-galactosidase.	2002-02-15	11787015
Evidence for a central inhibitory role of growth hormone secretagogues and ghrelin on gastric acid secretion in conscious rats.	2002-02	11867937
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
Gastric antisecretory and antiulcer activity of oxytocin in rats and guinea pigs.	2001-11-21	11764003
Adsorption properties and activities of lipase on a gold substrate modified by self-assembled monolayers.	2001-11	11791710
Synthesis and anti-melanoma activity of analogues of N-acetyl-4-S-cysteaminylphenol substituted with two methyl groups alpha to the nitrogen.	2001-02	11762644
Taurine and hypotaurine in spermatozoa and epididymal fluid of cats.	2001	11787195
Oxidative stress, glucose-6-phosphate dehydrogenase and the red cell.	2001	11765001
Cholesterol 7alpha-hydroxylase. 2. Biochemical properties and participation of endogenous cholesterol in the assay in vitro.	1975-06-16	1175606

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.

Substance Class	Chemical Created by `admin` on `Mon Mar 31 18:15:19 GMT 2025` Edited by `admin` on `Mon Mar 31 18:15:19 GMT 2025`
Record UNII	QO84GZ3TST
Record Status	`Validated (UNII)`
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Name

Type

Language

MERCAPTAMINE BITARTRATE

Preferred Name

English

CYSTEAMINE BITARTRATE

Common Name

English

MERCAMINE BITARTRATE

Common Name

English

CYSTEAMINE BITARTRATE [ORANGE BOOK]

Common Name

English

Mercaptamine bitartrate [WHO-DD]

Common Name

English

CYSTAGON

Brand Name

English

CYSTEAMINE BITARTRATE [JAN]

Common Name

English

ETHANETHIOL, 2-AMINO-, (2R,3R)-2,3-DIHYDROXYBUTANEDIOATE (1:1)

Systematic Name

English

MERCAPTAMINE BITARTRATE [MART.]

Common Name

English

RP-103

Code

English

.BETA.-MERCAPTOETHYLAMINE HYDROGEN TARTRATE

Systematic Name

English

CYSTEAMINE BITARTRATE [MI]

Common Name

English

MERCAPTOETHYLAMINE BITARTRATE

Systematic Name

English

CYSTEAMINE BITARTRATE [VANDF]

Common Name

English

PROCYSBI

Brand Name

English

Classification Tree Code System Code

NCI_THESAURUS

C29701