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The assessment and treatment of the musculoskeletal manifestations of cystinosis. [PDF]
Front NephrolSingh P, Marsh D, Sharkey M.
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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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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, 1968Cysteamine 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 +4 more
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Adrenergic blocking action of cysteamine
Experientia, 1962Il 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 +3 more
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Treatment of cystinosis with cysteamine
The Journal of Pediatrics, 1979C 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 +2 more
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Understanding intestinal cysteamine bitartrate absorption
The Journal of Pediatrics, 2006To 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 +6 more
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