cystinosis

Cysteamine hydrochloride is an orally administered small-molecule drug commonly used for the treatment of nephropathic cystinosis. It also functions as an antioxidant and is used in experimental studies to induce gastrointestinal disorders, duodenal ulcers, and kidney injury models in mice.

L-Cystine disodium monohydrate is the orally active extracellular form of L-Cysteine found in plant and animal proteins. It enhances the expression of the Nrf2 protein and activates the Nrf2 transcription factor. Additionally, L-Cystine disodium monohydrate decreases the production of reactive oxygen species (ROS) and safeguards cells from oxidant or Doxorubicin-induced apoptosis. When used with L-theanine, it boosts the production of antigen-specific IgG by increasing glutathione (GSH) levels and Th2-mediated responses. L-Cystine disodium monohydrate shows potential for research into cystinosis and kidney stones.

Cysteamine-D4 hydrochloride is the deuterated form of cysteamine hydrochloride. Cysteamine hydrochloride (T6460) (2-Aminoethanethiol hydrochloride) is an orally administrable small molecule active compound used to treat nephropathic cystinosis and also serves as an antioxidant.