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1 captopropionic acid, 2-mercapto ethanol, and cysteamine).
2 iers such as SIGCAFKILGY(-cysteamine) [SIGC(-cysteamine)].
3 to produce pantothenic acid (vitamin B5) and cysteamine.
4 increased the production of hypotaurine from cysteamine.
5 backbone derived from HCN and branches from cysteamine.
6 mines which can be driven by ADO's substrate cysteamine.
7 o biologically relevant thiols: cysteine and cysteamine.
8 pionic acid, 4-pyridinylethanemercaptan, and cysteamine.
9 caftor and/or the alternative CFTR modulator cysteamine.
11 terms of decreasing corneal cystine density, cysteamine (0.55%) was better than cystamine (0.55%), an
12 tify a thiol oxidase, previously assigned as cysteamine (2-aminoethanethiol) dioxygenase (ADO), as a
14 rystals, P21/n space group) using zwitterion cysteamine (2-aminoethanethiol) linker, and its remarkab
15 -cysteine with isosteric substrate analogues cysteamine, 3-mercaptopropionic acid, and propane thiol
18 pered our understanding of the metabolism of cysteamine, a product of the constitutive degradation of
19 pporting this model, maternal treatment with cysteamine-a lysosome-penetrant thiol-rescued the develo
20 ation and ligation with molecules containing cysteamine, analogous to native chemical ligation of thi
21 DO) is a thiol dioxygenase that sulfinylates cysteamine and amino-terminal cysteines in polypeptides.
22 herapy with a cystine-depleting drug such as cysteamine and an mTOR pathway inhibitor such as everoli
25 of cystinotic iPSCs or kidney organoids with cysteamine and everolimus corrects all of the observed p
27 However, experimental studies with the thiol cysteamine and its disulfide cystamine have demonstrated
29 ostasis in humans by oxidizing the sulfur of cysteamine and N-terminal cysteine-containing proteins t
30 nol formation, while mixed SAMs with varying cysteamine and propanethiol ratios allow for precise tun
31 e or withanolide A, was highly reactive with cysteamine and rapidly succumbed to irreversible nucleop
32 ctroscopies, we explored the binding mode of cysteamine and RGS5 to human and mouse ADO proteins in t
33 therefore studied the actions of cystamine, cysteamine and several reference thiol agents as cytopro
34 ences in reactivity of thiocyanurates toward cysteamines and thiols has been explained based on conce
35 ell-known radioprotective agents L-cysteine, cysteamine, and 2-[(aminopropyl)amino]ethanethiol (WR-10
42 t assay using 14C-(C-1)-labeled L-serine and cysteamine as substrates, counting the thialysine produc
44 ndings suggest that combination therapy with cysteamine bitartrate and N-acetylcysteine is associated
45 aimed to assess whether combination of oral cysteamine bitartrate and N-acetylcysteine is beneficial
46 is required to determine safe and effective cysteamine bitartrate concentrations to further evaluate
48 olled trial to determine whether 52 weeks of cysteamine bitartrate delayed release (CBDR) reduces the
49 d liver histology using data from the CyNCh (Cysteamine Bitartrate Delayed-Release for the Treatment
52 cally evaluated the therapeutic potential of cysteamine bitartrate in RC disease models spanning thre
56 tic treatment of surf1-/- larvae with either cysteamine bitartrate or N-acetylcysteine, but not other
60 s, we evaluated the antifibrotic efficacy of cysteamine bitartrate, an antioxidant therapy for patien
61 emonstrated the narrow therapeutic window of cysteamine bitartrate, with toxicity at millimolar level
62 ologues with O(2) - as in case of the parent cysteamine-bound complexes - the dioxygenation of the ch
64 e vanin-1 produces pantothenic acid (PA) and cysteamine, but the role of the vanin-1 /PA axis in meta
65 the sensitive voltammetric determination of cysteamine (CA), nicotinamide adenine dinucleotide (NADH
66 such as acetone, acetaldehyde, isoprene, or cysteamine can be detected in the breath gas with SPI, R
74 Lipophilic thiols such as mercaptoethanol or cysteamine could partially reverse the CCl4-induced calc
78 yer principle by modifying Au electrode with cysteamine (Cys) and immobilization of ferrocene cored p
79 lized on the Au/SPE previously aminated with cysteamine (Cys) by self-assembling monolayer technique.
82 newer formulation, the standard formulation (cysteamine [Cystaran]; 0.55% cysteamine hydrochloride +
83 nic acid (cysteine without the amino group), cysteamine (cysteine without the carboxylic acid), or me
91 etal-substituted catalytic activity of human cysteamine dioxygenase (ADO), an enzyme pivotal in regul
94 ly of enzymes includes cysteine dioxygenase, cysteamine dioxygenase, mercaptosuccinate dioxygenase, a
96 ependent dioxygenase 2-aminoethanethiol (aka cysteamine) dioxygenase (ADO) has recently been identifi
97 ned carbon chain and lacks a carboxyl group, cysteamine displays a catalytic efficiency (kcat/Km) wit
99 nolayers including mercaptobenzimidazole and cysteamine films, showcasing the potential for tailoring
100 modified with a self-assembled monolayer of cysteamine followed by cross-linking with glutaraldehyde
102 to be ineffective at zinc ejection, although cysteamine formed a transient complex with the zinc fing
103 d that the steady state percentage yields of cysteamine from cystamine and pantethine during the tran
105 In the case of the bacterial member ELIC, a cysteamine-gated channel from Erwinia chrisanthemi, a st
106 abeled diketide-SNAC 2 and N-[1-(14)C-acetyl]cysteamine gave a k(exch) of 0.15 +/- 0.06 min(-)(1), wi
108 amine) specifically and efficiently leads to cysteamine (half-cystamine) modification of a single sit
109 ted the extracellular levels of free reduced cysteamine, homocysteine, and cysteine from OHSCs within
112 rd formulation (cysteamine [Cystaran]; 0.55% cysteamine hydrochloride + benzalkonium chloride 0.01%)
116 sed on layer-by-layer assembly was formed by cysteamine in combination with a fourth-generation poly(
117 relies upon the finding that hCBS will take cysteamine in place of L-homocysteine, thereby producing
121 of N-acetylating a model compound containing cysteamine in the presence of acetyl-CoA, consistent wit
122 we investigate the reaction between HCN and cysteamine in water, which exhibits both sigmoidal react
123 eptide, and subsequent Michael addition with cysteamine increased masses by the predicated 77 and 154
126 eatest when combined with CFTR modulators or cysteamine, justifying further clinical testing of (R)-r
127 ng either a protein A sensor chip (SPR) or a cysteamine layer that modified the gold crystal (QCM-D)
128 bilayer and semi circled DDA on the MPA and cysteamine layers were confirmed by the increased redox
131 detection/quantification method that couples cysteamine-mediated HCHO scavenging with SPME GC-MS anal
132 opamine by the RNA aptamer, immobilized at a cysteamine-modified Au electrode, and further electroche
133 interactions between the positively charged cysteamine-modified electrode and the negatively charged
135 be performed by the RNA aptamer tethered to cysteamine-modified gold electrodes via the alkanethiol
138 , heme oxygenase-1, neutrophil infiltration, cysteamine, mucin, hydrogen sulfide, ghrelin, adiponecti
139 e chain elongation intermediates as N-acetyl cysteamine (NAC) thioesters and have used them as substr
141 is work confirms the effectiveness of copper-cysteamine nanoparticles as a photosensitizer when activ
143 n this paper, we report on the use of copper-cysteamine nanoparticles, designed to be targeted to tum
144 se treatment with the cystine-depleting drug cysteamine only slows disease progression, there is an u
145 e synthesis of taurine, the final product of cysteamine oxidation and the second most abundant amino
148 ved when bronchial epithelia were exposed to cysteamine plus the antioxidant food supplement EGCG.
149 rophage cultures revealed that cystamine and cysteamine possess significant antiviral properties at n
150 with 70 mol % mercaptopropanol and 30 mol % cysteamine/propanedithiol to facilitate membrane fusion
152 ults, treatment of cultured macrophages with cysteamine reduced cellular generation of reactive oxyge
153 rs (e.g., DMSO, glycerol, and cationic thiol cysteamine) reduces the incidence of instability after i
154 m and cystamine, diethyl dithiocarbamate and cysteamine, respectively, were found to be ineffective a
155 erestingly, exposure of cells to cysteine or cysteamine resulted in elevated intracellular hypotaurin
156 cystinotic cells' cystine content by use of cysteamine results in normalization of the apoptotic rat
157 peptides 2a-c and 3a-c to the reactive thiol cysteamine revealed that DeltaAla-containing peptides un
159 pecifically, Cu substrates are modified with cysteamine SAMs to uniformly anchor a Au(3+)-dimercaptos
163 unosensor for the detection of MDM2 based on cysteamine self assembled monolayers on a clean polycrys
167 calls into question the autophagy modulator cysteamine, since no rescue of mutant CFTR function was
169 ite-directed mutagenesis indicate that SIGC(-cysteamine) specifically and efficiently leads to cystea
170 e and the anti-oxidants allantoin, anserine, cysteamine, spermine, and squalene were amongst those on
171 od an aptamer was used to aggregate cationic cysteamine-stabilized CdTe/ZnS core/shell quantum dots,
172 Other antioxidants, approved for human use (cysteamine, succimer, dimercaprol), were not efficacious
173 utants in the presence of a concentration of cysteamine that elicits an intracluster open probability
176 able analysis included delayed initiation of cysteamine therapy and higher mean leucocyte cystine lev
180 In a study of renal ischemia reperfusion, cysteamine therapy initiated 10 days after injury and co
183 de that serves as a chemical intermediate in cysteamine therapy of cystinosis, and PQLC2 gene silenci
184 adulthood and the effects of long-term oral cysteamine therapy on its nonrenal complications have no
185 patients with cystinosis who were receiving cysteamine therapy were recruited from three European re
186 who received long-term (> or =8 years) oral cysteamine therapy were taller and heavier, had a renal
188 eaction of some of its epoxide moieties with cysteamine to afford a monolith rich in surface thiol gr
189 hrough reaction of its epoxide moieties with cysteamine to afford a monolith rich in surface thiol gr
191 g was captured by the electrode surface with cysteamine to detect the analyte, IL-6, after being atta
196 displayed more severe lesions in response to cysteamine treatment compared with wild-type counterpart
197 othyroidism, and death increased as time off cysteamine treatment increased, and it decreased as time
199 viously unrecognized antifibrotic actions of cysteamine via TGF-beta-independent mechanisms that incl
200 ne) decorated with gold nanoparticles-capped cysteamine was fabricated on the surface of gold electro
202 phosphopantetheine, pantetheine, and finally cysteamine was observed with ThnR, ThnH, and ThnT, respe
203 onyl-S-pantetheine or L-threonyl-S-(N-acetyl)cysteamine was used as a small-molecule thioester analog
204 st successful of these biomimetic catalysts (cysteamine) was used to encapsulate firefly luciferase,
205 athione, and the simplest stable aminothiol, cysteamine, we enabled the nanoparticles to exhibit not
206 ead-space extraction SPME-GC-MS method using cysteamine, which provides similarly sensitive HCHO quan
207 nction was detected following treatment with cysteamine, while deleterious effects were observed when
208 s and randomized controlled trials comparing cysteamine with control or other formulations for treatm
209 2+/-0.2 mM for L-serine and 5.6+/-2.2 mM for cysteamine, with kcat = 1.3+/-0.1s(-1) for the formation