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Cysteamine and cystamine

1987
Publisher Summary This chapter discusses about cysteamine and cystamine. Cysteamine (2-aminoethanethiol) is formed in mammalian tissues by the enzymatic hydrolysis of pantetheine, and has the role in the biosynthesis of hypotaurine. Cysteamine has been used therapeutically to treat cystinosis and sickle cell anemia, and has been used as a protective ...
M W, Duffel, D J, Logan, D M, Ziegler
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Metal Content of Cysteamine Oxygenase

European Journal of Biochemistry, 1968
Cysteamine oxygenase has been analysed for the presence of metals, by spectrographic analysis, and X‐ray fluorescence. Iron, copper, and zinc have been found in the enzyme in the quantity accounting for 1 atom of each metal per molecule of enzyme.Passage of the enzyme through a chelating resin column did not appreciable change the specific activity but
D, Cavallini, S, Duprè, R, Scandurra, M T, Graziani, F, Cotta-Rasusino   +4 more
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Adrenergic blocking action of cysteamine

Experientia, 1962
Il a ete demontre que la cysteamine provoque une activation centrale adrenergique accompagnee par une liberation des catecholamines de la surrenale. Plus tard cet effet est suivi par un blocage periferique des recepteurs adrenergiques.
R, DEBIJADJI, V, VARAGIC, S, ELCIC, J, DAVIDOVIC   +3 more
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Treatment of cystinosis with cysteamine

The Journal of Pediatrics, 1979
C v s T I N 0 S I S is a recessively inherited metabolic disorder characterized by an accumulation of nonprotein cystine within the lysosomes. 1 ! n the juvenile nephropathic form, the disease presents with tubular renal dysfunction and progressive glomerular damage, leading to end-stage renal failure and eventual death.
E P, Girardin, M S, DeWolfe, J F, Crocker   +2 more
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Understanding intestinal cysteamine bitartrate absorption

The Journal of Pediatrics, 2006
To test the hypothesis that a controlled-release preparation of cysteamine, with fewer daily administrations, would improve the quality of life for patients with cystinosis.A specifically designed nasoenteric tube was used to administer cysteamine directly into the stomach, small intestine (SI) and colon and serial plasma cysteamine, serum gastrin and ...
Ranjan, Dohil, Meredith, Fidler, Bruce A, Barshop, Jon, Gangoiti, Reena, Deutsch, Michael, Martin, Jerry A, Schneider   +6 more
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Cysteamine

Drug Discovery Today, 2013
Cysteamine is an amino thiol with the chemical formula HSCH2CH2NH2. 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 ...
Besouw, Martine, Masereeuw, R., van den Heuvel, Lambert, Levtchenko, Elena   +3 more
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Cysteamine Toxicity in Patients with Cystinosis

The Journal of Pediatrics, 2011
To report new adverse effects of cysteamine.Detailed clinical information was obtained from the patients' physicians.New adverse events were reported in 8 of 550 patients with cystinosis treated with cysteamine in Europe during the last 5 years. Detailed clinical information was not available for 2 of these patients, 1 of whom died from cerebral ...
Besouw, M.T., Bowker, R., Dutertre, J.P., Emma, F., Gahl, W.A., Greco, M., Lilien, M.R., McKiernan, J., Nobili, F., Schneider, J.A., Skovby, F., van den Heuvel, L.P., van den Heuvel, L.P., van 't Hoff, W.G., Levtchenko, E.N.   +14 more
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Cysteamine-induced adrenocortical necrosis in rats

Experimental and Molecular Pathology, 1981
Abstract The development of adrenocortical hemorrhagic necrosis following treatment with cysteamine was studied in rats. Increased vascular permeability, originating in the inner cortical layers, resulted in congestion, platelet aggregation, edema, and hemorrhage which, over a 12-hr period, spread outward to the capsule.
D J, McComb, K, Kovacs, H C, Horner, G T, Gallagher, U, Schwedes, K H, Usadel, S, Szabo   +6 more
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Unveiling thiol biomarkers: Glutathione and cysteamine

Clinica Chimica Acta
The physiological and clinical importance of Glutathione and Cysteamine is emphasized by their participation in a range of conditions, such as diabetes, cancer, renal failure, Parkinson's disease, and hypothyroidism. This necessitates the requirement for accessible, expedited, and cost-efficient testing that can facilitate clinical diagnosis and ...
M G, Gopika, Surya, Gopidas, Gokul S, Jayan, P S, Arathy, Beena, Saraswathyamma   +4 more
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