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1 isease was negatively correlated with plasma cystine).
2 ls (e.g., l-cysteine) into disulfides (e.g., cystine).
3 antioxidant role by exporting glutamate for cystine.
4 etal-organic frameworks, calcite, urea and l-cystine.
5 uria by increasing the solubility of urinary cystine.
6 ysosomal accumulation and crystallization of cystine.
7 ystinosin rather than to the accumulation of cystine.
8 detecting and quantitating insoluble urinary cystine.
9 ntiporter that exports glutamate and imports cystine.
10 alyze the aerobic oxidation of l-cysteine to cystine.
11 other pair of the plesiotypic LU domain half-cystines.
12 logical concentrations of cysteine (10 mum), cystine (50 mum) and glutamate (100 mum) in order to pre
13 ioxidant production through the provision of cystine, a key precursor in glutathione biosynthesis.
15 We find that levels of a single nutrient, cystine, accounts for the differential dependence on glu
17 increasing evidence supports the notion that cystine accumulation alone is not responsible for the en
18 lysosomal cotransporter cystinosin, leads to cystine accumulation and cellular damage in various orga
19 utations of the cystinosin gene CTNS lead to cystine accumulation and crystals at acidic pH in patien
20 only a Ctns knockout mouse reported, showing cystine accumulation and late signs of tubular dysfuncti
21 efective cystinosin function, intralysosomal cystine accumulation and the development of cystinosis.
22 ions, extrarenal organs may be affected from cystine accumulation even in childhood, especially in pa
23 that lysosomal storage triggered by soluble cystine accumulation induces apical PTC dedifferentiatio
25 is is a multisystemic disease resulting from cystine accumulation primarily in kidney and many other
28 to examine hallmarks of cystinosis-including cystine accumulation, lysosome size, the autophagy pathw
29 everest form, cystinosis is characterized by cystine accumulation, renal proximal tubule dysfunction,
30 n cystinotic mice efficiently blocked kidney cystine accumulation, thereby preventing lysosomal defor
33 ctivating pre-existing oncogenic pathways in cystine-addicted TNBC with prominent mesenchymal feature
38 ndependent breast cancer cells conferred the cystine-addiction phenotype by modulating the signaling
39 addiction phenotype can be abrogated in the cystine-addictive cells by miR-200c, which converts the
40 To test this hypothesis, we used the toxic cystine analogue selenocystine to initially characterize
42 ase (an engineered enzyme that degrades both cystine and cysteine) in combination with checkpoint blo
43 ulfidation in cells in response to exogenous cystine and evidence for the formation of polysulfides u
44 that added cysteine abiotically oxidizes to cystine and exponentially growing E. coli degrade high c
45 d transcriptional repression of system xc(-) cystine and glutamate antiporter via the JAK/STAT1 pathw
48 ced (cysteine and glutathione) and oxidized (cystine and glutathione disulphide) aminothiols were qua
50 llowing glucose deprivation, the import of l-cystine and its subsequent reduction to l-cysteine deple
53 effect of adding tetra-sodium pyrophosphate, cystine and lysine as surimi gelation enhancers (Alaska
54 ical properties of the gels were determined, cystine and lysine were found to be the most effective a
60 with this model, we found that the glutamate/cystine antiporter (xCT) is required for increased sensi
62 2 and SLC7A11, two subunits of the glutamate-cystine antiporter system x(c)(-), impairs the uptake of
65 ed to assess the effectiveness and safety of cystine as a smoking cessation aid in patients with tube
67 S. aureus acquires host-derived cysteine and cystine as sources of nutrient sulfur during systemic in
68 on and quantification of vitreous humor (VH) cystine as well as provide the portability for on spot d
69 establish CydDC as a reducer of cytoplasmic cystine, as opposed to an l-cysteine exporter, and furth
74 ngineered by introduction of a nonperturbing cystine bridge (FVIIa Q64C-sTF G109C) in the interface.
75 upied glycosylation sites and six intrachain cystine bridges with Cys-158 of the very flexible N-term
77 porter system x(c)(-), impairs the uptake of cystine by tumour cells, and as a consequence, promotes
78 there is a linear correlation between the VH cystine concentration and TSD as the concentration of cy
80 D (the dependent variable), RGB intensity of cystine concentration till 24h (the independent variable
81 ore rapid and frequent monitoring of urinary cystine concentration would significantly improve the di
83 ), in vivo confocal microscopy score (IVCM), cystine crystal depth, contrast sensitivity (CS), photop
84 nt or response to therapy, change in corneal cystine crystal score (CCCS), in vivo confocal microscop
85 y other crystalline inflammasome activators, cystine crystal-induced IL-1beta secretion required acti
88 vel, amorphous lysosomal inclusions preceded cystine crystals and eventual atrophy without crystals.
89 ed PTC apoptosis allowed luminal shedding of cystine crystals and was partially compensated for by tu
90 Taken together, these data demonstrate that cystine crystals are endogenous inflammasome-activating
91 to other host-derived crystalline moieties, cystine crystals can induce IL-1beta production through
94 ivating stimuli, suggesting a novel role for cystine crystals in the pathogenesis of nephropathic cys
100 n which sulfide produced by CdsH reacts with cystine (Cys-S-S-Cys), S-sulfocysteine (Cys-S-SO3 (-)),
101 dox systems including extracellular cysteine/cystine (Cys/CySS), intracellular glutathione/oxidized g
102 d to proliferate in medium supplemented with cystine, cysteine, or N-acetyl cysteine as the sole sulf
105 t(e)inase, a drug that depletes cysteine and cystine, demonstrating a translatable means to induce fe
106 the platform's capabilities by identifying a cystine-dense peptide capable of inhibiting the YAP:TEAD
107 herapeutics is nature-inspired bioengineered cystine-dense peptides (CDPs) for various biological tar
110 be a platform for identifying target-binding cystine-dense peptides using mammalian surface display,
111 platform provides the opportunity to screen cystine-dense peptides with drug-like qualities against
116 ions suggest that combination therapy with a cystine-depleting drug such as cysteamine and an mTOR pa
117 op new treatments not dependent on lysosomal cystine depletion alone for this devastating disease.
124 genetic and mechanistic basis to explain how cystine deprivation triggers necrosis by activating pre-
128 enotype is associated with a higher level of cystine-deprivation signatures noted in the basal type b
131 and twenty-four times more effective than l-cystine dimethyl ester (CDME) in increasing the metastab
132 aracterized by defective lysosomal efflux of cystine due to mutations in the CTNS gene encoding the l
134 densation of two nontoxic building blocks: L-cystine ester and versatile fatty diacids, which make th
135 p2-KO GCPs, the levels of both the glutamate-cystine exchanger Sc7a11 and glutathione were increased;
139 e for transporting the disulphide amino acid cystine from the lysosomal compartment into the cytosol.
143 RNA sequencing revealed that inhibition of cystine-glutamate exchange leads to activation of an ER
145 contrast, pharmacological inhibition of the cystine/glutamate antiporter dramatically attenuated isc
146 h control animals, mice lacking a functional cystine/glutamate antiporter exhibited reduced anoxic de
151 zymes was unchanged, xCT, a component of the cystine/glutamate antiporter system x(c)(-), was signifi
152 dentified xCT, the functional subunit of the cystine/glutamate antiporter system xc(-), as a surface
153 System xc(-) is a heteromeric amino acid cystine/glutamate antiporter that is constitutively expr
154 genes, including SLC7A11, a component of the cystine/glutamate antiporter that regulates reactive oxy
156 SLC7A11), the light chain of system xc-, the cystine/glutamate antiporter, suggests that PCP also reg
159 Here we explored the potential of targeting cystine/glutamate exchanger (SLC7A11/xCT), which contrib
160 he JCI, Soria and colleagues reveal that the cystine/glutamate exchanger is an important source of ex
162 was located in SLC7A11A, a gene involved in cystine/glutamate transport and the biosynthesis of glut
163 g substrate in glutathione biosynthesis, the cystine/glutamate transporter (system xc(-)) represents
164 tress, quantified by the plasma aminothiols, cystine, glutathione, and their ratio, is associated wit
166 ludes product inhibition of the importer, so cystine import continues into cells that are already sat
174 ATR-FTIR) to detect and quantitate insoluble cystine in 22 cystinuric and 5 healthy control urine sam
175 e (SeMet), methyl-seleno-cysteine and seleno-cystine in extra-virgin olive oil (EVOO) was developed a
176 , loss of MFSD12 reduced the accumulation of cystine in lysosomes of fibroblasts from patients with c
177 de-rich plasma proteins could be a source of cystine in proximal tubular cells, we used a mouse model
178 cystinosis, characterized by accumulation of cystine in the lysosomes, is caused by mutations in CTNS
179 ound that reintroducing the two missing half-cystines in uPAR DI caused the spontaneous formation of
181 ntrast, luminal-type breast cancer cells are cystine-independent and exhibit little death during cyst
182 f epithelial-mesenchymal transition (EMT) in cystine-independent breast cancer cells conferred the cy
188 xpression, in conjunction with environmental cystine, is necessary and sufficient to increase glutami
191 the toxin comprises a well-defined inhibitor cystine knot (ICK) backbone region and a flexible C-term
193 ins: N-TRTX-Preg1a, exhibiting an inhibitory cystine knot (ICK) motif, and N-BUTX-Ptr1a, a short scor
196 ion to synthesize dimers of integrin-binding cystine knot (knottin) miniproteins with low-picomolar b
199 n together, our results expand membership of cystine knot alpha-amylase inhibitors in the Apocynaceae
202 harmacologically active C-C-CC-C-C inhibitor cystine knot and CC-C-C motifs (168 and 44 toxins, respe
204 that Hi1a comprises two homologous inhibitor cystine knot domains separated by a short, structurally
207 ed by their head-to-tail cyclic backbone and cystine knot motif, which render them to be exceptionall
211 ribe the engineering and validation of a new cystine knot peptide (knottin) that selectively recogniz
212 toxin guangxitoxin-1E (GxTX), an inhibitory cystine knot peptide that binds selectively to Kv2-type
215 e V-ATPase of pea albumin 1b (PA1b), a small cystine knot protein that shows exquisitely selective in
218 ions from several venoms and characterized a cystine knot toxin called JZTx-27 from the venom of tara
219 hypothesize parallel evolution of inhibitor cystine knot toxins from Araneomorphae and Mygalomorphae
221 was found to adopt the so-called "inhibitor cystine knot" or "knottin" fold stabilized by three disu
222 it potentiates formation of the beta-subunit cystine knot, and second, contacts between alpha-subunit
225 uvian green velvet tarantula is an inhibitor cystine-knot peptide and selective antagonist of the hum
229 We have found that the structurally related cystine-knot protein, nerve growth factor beta (NGFbeta)
230 is a neuropeptide hormone consisting of two cystine-knot proteins (burs alpha and burs beta), respon
232 pectroscopy revealed that roseltide rT7 is a cystine-knotted, six-cysteine hevein-like cysteine-rich
233 t the substrate-binding protein FliY binds l-cystine, l-cysteine, and d-cysteine with micromolar affi
234 We evaluated mice aged 6-9 months for kidney cystine levels and crystals; histopathology, with emphas
237 independent predictors for white blood cell cystine levels in patients of all ages with cystinosis;
238 cations and was superior to white blood cell cystine levels in predicting the presence of multiple ex
239 ic control (on the basis of white blood cell cystine levels of <2 nmol 1/2 cystine/mg protein or >=2
240 ls, these cystinosis models exhibit elevated cystine levels, increased apoptosis, and defective basal
243 entify a coupling between SLC7A11-associated cystine metabolism and the pentose phosphate pathway, an
246 Amino acid analysis gave high levels of cystine/methionine, histidine and tyrosine/phenylalanine
247 with FAO/WHO recommended pattern except for cystine/methionine, isoleucine, tyrosine/phenylalanine,
248 ite blood cell cystine levels of <2 nmol 1/2 cystine/mg protein or >=2 nmol 1/2 cystine/mg protein, r
250 cation of novel flower and palm-leaf like 3D cystine microstructures (CMs) with high uniformity havin
251 mprinted polymer two biocompatible monomers (cystine monomer and N-vinyl caprolactam) were used, whic
254 with either lower cellular concentrations of cystine or glutamate, despite opposing the transport of
258 disorder characterized by defective urinary cystine reabsorption that results in the formation of cy
259 tammetric current responses for the cysteine-cystine redox cycle in nondegassed aqueous buffer media
260 ults in marked accumulation of intracellular cystine, redox system collapse and rapid cell death, whi
261 ng the metastable supersaturation range of l-cystine, respectively, effectively inhibiting l-cystine
262 correlated with a depletion of intracellular cystine resulting from increased de novo glutathione bio
265 dSTP can accurately identify a wide range of cystine stabilized peptide toxins directly from sequence
266 tional supplement alpha-lipoic acid inhibits cystine stone formation in the Slc3a1(-/-) mouse model o
267 can readily form microcrystals that lead to cystine stone formation, especially at low urine pH.
268 that results from the acquisition and use of cystine, the oxidized form of cysteine, as a source of c
269 D12 is required to maintain normal levels of cystine-the oxidized dimer of cysteine-in melanosomes, a
273 regulation of SLC7A11 augments intracellular cystine transport and increases intracellular levels of
277 results show a dual role for cystinosin as a cystine transporter and as a component of the mTORC1 pat
278 tions in the gene that encodes the lysosomal cystine transporter cystinosin cause the lysosomal stora
280 t chemotherapy induces the expression of the cystine transporter xCT and the regulatory subunit of gl
283 epends on 3MST, whereas the CysB-regulated l-cystine transporter, TcyP, plays the principle role in t
285 m urate, calcium pyrophosphate dihydrate and cystine trigger caspase-independent cell death in five d
289 rface transport system xC(-), which mediates cystine uptake, a pivotal step in glutathione synthesis
290 etion of xCT in tumor cells led to defective cystine uptake, accumulation of reactive oxygen species,
291 (mTOR) kinase, promotes glutamate secretion, cystine uptake, and incorporation into glutathione, link
292 Down-regulation of CD44/CD44v8-10 impaired cystine uptake, lowered intracellular reduced glutathion
293 e show that the import of oxidized cysteine (cystine) via system x(C) (-) is a critical dependency of
298 a high enzymatic activity in reduction of l-cystine, where the catalytic efficiency (2,217 min(-1)mi
300 sulfasalazine, an FDA-approved inhibitor of cystine xCT antiporter, in culture and xenograft assays.