Cystinuria: genetic aspects, mouse models, and a new approach to therapy
Sign Up to like & getrecommendations! Published in 2018 at "Urolithiasis"
DOI: 10.1007/s00240-018-1101-7
Abstract: Cystinuria, a genetic disorder of cystine transport, is characterized by excessive excretion of cystine in the urine and recurrent cystine stones in the kidneys and, to a lesser extent, in the bladder. Males generally are… read more here.
Keywords: mouse models; cystine; cystinuria genetic; new approach ... See more keywords
Extracellular cystine influences human preadipocyte differentiation and correlates with fat mass in healthy adults
Sign Up to like & getrecommendations! Published in 2021 at "Amino Acids"
DOI: 10.1007/s00726-021-03071-y
Abstract: Plasma cysteine is associated with human obesity, but it is unknown whether this is mediated by reduced, disulfide (cystine and mixed-disulfides) or protein-bound (bCys) fractions. We investigated which cysteine fractions are associated with adiposity in… read more here.
Keywords: extracellular cystine; influences human; cystine; mass healthy ... See more keywords
Cystinosis: A 6.5-Year-Follow-up Study
Sign Up to like & getrecommendations! Published in 2022 at "Indian Journal of Pediatrics"
DOI: 10.1007/s12098-022-04161-9
Abstract: To the Editor: Cystinosis is an autosomal recessive disorder, in which, free cystine accumulates continuously in lysosomes throughout the body [1]. We diagnosed cystinosis when proximal renal tubular acidosis (PRTA), with or without Fanconi syndrome… read more here.
Keywords: ckd stage; year follow; follow study; cystinosis ... See more keywords
Oxidative stress response of Deinococcus geothermalis via a cystine importer
Sign Up to like & getrecommendations! Published in 2017 at "Journal of Microbiology"
DOI: 10.1007/s12275-017-6382-y
Abstract: A cystine-dependent anti-oxidative stress response is characterized in Deinococcus geothermalis for the first time. Nevertheless, the same transcriptional directed Δdgeo_1985F mutant strain was revealed to have an identical phenotype to the wild-type strain, while the… read more here.
Keywords: cystine; mutant strain; strain; wild type ... See more keywords
Transport of cystine across xC- antiporter.
Sign Up to like & getrecommendations! Published in 2019 at "Archives of biochemistry and biophysics"
DOI: 10.1016/j.abb.2019.01.039
Abstract: Extracellular cystine (CYC) uptake by xC- antiporter is important for the cell viability. Especially in cancer cells, the upregulation of xC- activity is observed, which protects these cells from intracellular oxidative stress. Hence, inhibition of… read more here.
Keywords: cyc; cystine; transport; across antiporter ... See more keywords
In vitro and ex vivo implantation of cystine crystals and treatment by contact lens
Sign Up to like & getrecommendations! Published in 2019 at "Colloids and Surfaces A: Physicochemical and Engineering Aspects"
DOI: 10.1016/j.colsurfa.2018.11.031
Abstract: Abstract Cystinosis is a rare genetic disorder order affecting the transport of the amino acid cystine across the lysosomal membrane, leading to crystal formation throughout the body. The formation of these crystals in the cornea… read more here.
Keywords: cystine crystals; bioavailability; vitro vivo; cystine ... See more keywords
An automated and efficient conformation search of L-cysteine and L,L-cystine using the scaled hypersphere search method
Sign Up to like & getrecommendations! Published in 2017 at "Chemical Physics Letters"
DOI: 10.1016/j.cplett.2017.07.029
Abstract: Abstract Stable conformers of l -cysteine and l,l -cystine were explored using an automated and efficient conformational searching method. The Gibbs energies of the stable conformers of l -cysteine and l,l -cystine were calculated with… read more here.
Keywords: automated efficient; search; cysteine cystine; cystine ... See more keywords
Enhanced photocatalytic performance in preyssler-type P5W30CdS nanohybrids synthesized by l-cystine-mediated hydrothermal assembly
Sign Up to like & getrecommendations! Published in 2019 at "International Journal of Hydrogen Energy"
DOI: 10.1016/j.ijhydene.2019.03.214
Abstract: Abstract Nanohybrids of P5W30 CdS with intimate contact have been synthesized via a facile hydrothermal strategy in presence of l -cystine as sulfur precursor, chelating and anchoring linker molecule. In this process, nanosized CdS can… read more here.
Keywords: photocatalytic performance; cystine; preyssler type;
Target Recognition-Triggered Peroxidase-Mimicking Activity Depression in Homochiral Nanochannels for Identifying Cystine Enantiomers.
Sign Up to like & getrecommendations! Published in 2023 at "Analytical chemistry"
DOI: 10.1021/acs.analchem.3c00280
Abstract: Enantioselective identification of chiral molecules is of paramount importance in medical science, biochemistry, and pharmaceutics owing to the configuration-dependent activities of enantiomers. However, the identical physicochemical properties of enantiomers remain challenging in chiral sensing. In… read more here.
Keywords: recognition; mimicking activity; peroxidase mimicking; cystine ... See more keywords
Posterior Segment Involvement in Infantile Nephropathic Cystinosis - A Review.
Sign Up to like & getrecommendations! Published in 2023 at "Klinische Monatsblatter fur Augenheilkunde"
DOI: 10.1055/s-0037-1599653
Abstract: Cystinosis is a rare lysosomal storage disease with a prevalence of 1 : 100 000 - 1 : 200 000 cases. It is caused by biallelic mutations in the CTNS gene, which encodes cystinosin, that transport cystine out of the lysosomes. Due to… read more here.
Keywords: posterior segment; cystine crystals; cystine; review ... See more keywords
Tissue distribution, hormonal regulation, ontogeny, diurnal expression, and induction of mouse cystine transporters Slc3a1 and Slc7a9
Sign Up to like & getrecommendations! Published in 2020 at "Free Radical Research"
DOI: 10.1080/10715762.2020.1812597
Abstract: Abstract Slc7a11 (xCT) and Slc3a1 (rBAT) are cystine uptake transporters that maintain intracellular concentrations of cysteine, the rate-limiting amino acid in glutathione synthesis. This study was conducted to first determine the tissue distribution of the… read more here.
Keywords: tissue distribution; cystine; ontogeny diurnal; expression ... See more keywords