Synonyms: CI-9148 | Cystagon® | Cystaran® | cysteamine hydrochloride | L-1573
mercaptamine is an approved drug (FDA (1994), EMA (1997))
Compound class:
Synthetic organic
Comment: Mercaptamine is the simplest stable aminothiol, and a degradation product of the amino acid cysteine.
Ligand Activity Visualisation ChartsThese are box plot that provide a unique visualisation, summarising all the activity data for a ligand taken from ChEMBL and GtoPdb across multiple targets and species. Click on a plot to see the median, interquartile range, low and high data points. A value of zero indicates that no data are available. A separate chart is created for each target, and where possible the algorithm tries to merge ChEMBL and GtoPdb targets by matching them on name and UniProt accession, for each available species. However, please note that inconsistency in naming of targets may lead to data for the same target being reported across multiple charts. ✖ |
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No information available. |
Summary of Clinical Use |
The bitartrate and hydrochloride salt forms are indicated for the treatment of neuropathic cystinosis, and to treat radiation sickness. Link to ClinicalTrials.gov to view currently registered trials assessing efficacy of this drug for additional indications. |
Mechanism Of Action and Pharmacodynamic Effects |
The inherited lysosomal storage disease, cystinosis is characterized by the abnormal accumulation of the amino acid cystine and arises through inheritance of mutations in the lysosomal cystine transporter gene (CTNS, O60931). Intracellular cystine accumulation and crystallization causes widespread tissue damage. The free thiol group of cysteamine cleaves the disulphide bond in cystine, thereby allowing excretion of the cystine byproducts. |
External links |
For extended ADME data see the following: Electronic Medicines Compendium (eMC) European Medicines Agency (EMA) |