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(Mercaptoethyl)ammonium Hydrogen Tartrate;cysteamine Bitartrate;cystagon;cysteamine Bitratrate;2-Aminoethanethiol (2r,3r)-2,3-Dihydroxysuccinate;mercaptamine Bitartrate
Cysteamine Bitartrate Is an Aminothiol Salt Used in the Treatment of Nephropathic Cystinosis. Cysteamine Bitartrate Enters the Cell and Reacts with Cystine Producing Cysteine and Cysteine-Cysteamine Mixed Disulfide Compound, Both of Which, Unlike Cystine, Can Pass Through the Lysosomal Membrane. This Prevents the Accumulation of Cystine Crystals in the Lysosomes of Patients with Cystinosis, Which Can Cause Considerable Damage and Eventual Destruction of the Cells, Particularly in the Kidneys. (Nci05)
A Mercaptoethylamine Compound That Is Endogenously Derived from the Coenzyme a Degradative Pathway. The Fact That Cysteamine Is Readily Transported into Lysosomes Where It Reacts with Cystine to Form Cysteine-Cysteamine Disulfide and Cysteine Has LED to Its Use in Cystine Depleting Agents for the Treatment of Cystinosis.
| Storage conditions | Store at -20°C |
| Solubility | DMSO: 45 mg/mL (198.03 mM); Ethanol: insoluble |
| Water Solubility | Water: 45 mg/mL (198.03 mM) |
| InChI | InChI=1/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-;/s3 |
| InChIKey | NSKJTUFFDRENDM-RZHFEHKBNA-N |
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