Table of Contents
What is cysteamine hydrochloride used for?
Cysteamine is used to treat eye problems in people with cystinosis, a hereditary metabolic disorder that causes build-up of a certain natural substance (cystine) in the body. This medication helps get rid of cystine build-up in the eyes.
Where is cysteamine found?
Endogenously, cysteamine is derived from coenzyme A degradation, although its plasma concentrations are low. Most experience with cysteamine as a drug originates from the field of the orphan disease cystinosis, in which cysteamine is prescribed to decrease intralysosomal cystine accumulation.
Who makes cysteamine?
Horizon Therapeutics plc Announces U.S. FDA Acceptance of its New Drug Application to Make PROCYSBI® (Cysteamine Bitartrate) Available as Oral Granules in Packets.
Is cysteamine an antioxidant?
In addition to its role as an antioxidant, cysteamine is also known to inhibit the enzyme tissue transglutaminase 2 (Tgm2).
What is an Aminothiol?
The synthetic aminothiol, amifostine, originally developed as a radioprotector and earlier known as WR-2721 [S-2-(3-aminopropylamino) ethyl phosphorothioate], has been extensively used as a chemical radioprotector for the normal tissues in cancer radiotherapy and chemotherapy.
How does cysteamine work?
Cysteine is one of the few amino acids that contains sulfur . This allows cysteine to bond in a special way and maintain the structure of proteins in the body. Cysteine is a component of the antioxidant glutathione . The body also uses cysteine to produce taurine , another amino acid.
Is cysteamine a tyrosinase inhibitor?
In amelanotic cells, tyrosinase has a short half-life and is readily inhibited by cystamine/cysteamine whereas tyrosinase in the more mature melanosomes of the pigmented cell appears to be less accessible to proteolytic and thiol attack.
Is Cyspera safe during pregnancy?
Cysteamine cream should not be used by anyone with a personal or family history of the depigmenting disorder, vitiligo. Do no use if you are pregnant or breast-feeding.
Is Crystal Eye Drops FDA approved?
FDA approves crystal-dissolving eye drops, a major milestone for NIH rare disease researchers.
Why is cysteamine an antioxidant?
Cystamine and cysteamine have a variety of other effects which include antioxidant effects (by increasing cysteine and glutathione levels), inhibition of transglutaminase 2 and caspase 3 (possibly by modifying reactive cysteine residues or cysteaminylation), modulation of mitochondrial function, immunomodulation.