Cystinosis is a rare, autosomal recessive disorder leading to defective transport of cystine out of lysosomes. Subsequent cystine crystal accumulation can occur in various tissues, including the ocular surface. This review explores the efficacy of cysteamine hydrochloride eye drops in the treatment of corneal cystine crystal accumulation and its safety profile.

The chemotaxis system enables motile bacteria to search for an optimum level of environmental factors. Salmonella typhimurium senses the amino acid cysteine as an attractant and its oxidized dimeric form, cystine, as a repellent. We investigated the dose-response dependence of changes in chemotactic signaling activity upon exposure to cysteine and cystine of S. typhimurium LT2 using in vivo flu...

IN the ordinary putrefaction of protein material the protein-sulphur is liberated mainly as hydrogen sulphide accompanied in a few cases by relatively small amounts of mercaptans. The present paper is an account of an organism which is capable of the aerobic degradation of l-cystine with the direct liberation of free sulphur. In earlier work on the bacterial decomposition of cystine the medium ...

in this work, the waste stone sludge obtained from the granite and marble stone processing factories was used for the manufacture of artificial stones using vibratory compaction in a vacuum environment. the results obtained showed that water absorption and density increased, and the flexure, compressive, and tensile strengths decreased with increase in the content of the waste stone sludge. the...

An enzyme is described which, when coupled with glutathione reductase, catalyzes a thiol-disulfide interchange between glutathione and a number of disulfide substrates of low molecular weight. The most purified preparation is homogeneous on the basis of the disc gel electrophoresis test. From its behavior on a column of Sephadex G-75, the enzyme appears to have a molecular weight of about 15,00...

In the central nervous system, cystine import in exchange for glutamate through system xc- is critical for the production of the antioxidant glutathione by astrocytes, as well as the maintenance of extracellular glutamate. Therefore, regulation of system xc- activity affects multiple aspects of cellular physiology and may contribute to disease states. Pituitary adenylate cyclase-activating poly...

Published evidence indicates that cystine-containing proteins have their disulphide bonds reduced during proteolysis in lysosomes. However, the intralysosomal accumulation of cystine in the cells of patients with cystinosis has been seen as evidence that protein cystine residues are not reduced. The data are reconcilable and fully in harmony if it is postulated that cysteine from the cytoplasm ...

1. An intestinal perfusion technique has been used to study absorption in uivo of L-cystine and L-cysteine in six normal human subjects and three patients with homozygous cystinuria. 2. No significant absorption of L-cystine was detected during perfusion of the cystinuric patients, whereas L-cysteine absorption was normal. These results imply that L-cystine and L-cysteine are normally absorbed ...

5. The reaction kinetics of the hydrolytic decomposition of G2S2 and cystine in the presence of an excess of silver nitrate were studied under various conditions. The reactions were classified into three main types. 6. Acetate was shown to catalyse the reaction of cystine and, to a much lesser extent, that of. G2S2. Similarly, barbitone inhibited the cystine reaction completely, but that of G2S...