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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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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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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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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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Radiation Protection of Tissue Culture Cells by Anoxia, Cysteamine and a Combination of Anoxia and Cysteamine

Nature, 1962
Evidence is given in support of the hypothesis that the radioprotective action of a group of sulfhydryl compounds such as cysteine and cysteamine is not based on the removal of oxygen but is caused by another mode of action on the cellular level. In the experiments, cysteamine in concentrations of 4 and 16 mM- bar gave dose reduction factors of 1.8 and
A J, VERGROESEN, O, VOS, L, BUDKE
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