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Bin Liu et al.
Science (New York, N.Y.), 337(6092), 351-354 (2012-07-24)
Defective catabolite export from lysosomes results in lysosomal storage diseases in humans. Mutations in the cystine transporter gene CTNS cause cystinosis, but other lysosomal amino acid transporters are poorly characterized at the molecular level. Here, we identified the Caenorhabditis elegans
A circular dichroism sensor for selective detection of Cd2+ and S2- based on the in-situ generation of chiral CdS quantum dots
Sianglam P, et al.
Spectrochemical Analysis by Atomic Absorption and Emission, 183, 408-416 (2017)
Bahadur K C Remant et al.
Molecular pharmaceutics, 11(6), 1897-1905 (2014-05-02)
Ideal "smart" nanoparticles for drug delivery should enhance therapeutic efficacy without introducing side effects. To achieve that, we developed a drug delivery system (HCN) based on a polymer-drug conjugate of poly[2-(pyridin-2-yldisulfanyl)]-graft-poly(ethylene glycol) and camptothecin with an intracellularly cleavable linker and
Adrien Jézégou et al.
Proceedings of the National Academy of Sciences of the United States of America, 109(50), E3434-E3443 (2012-11-22)
Cystinosin, the lysosomal cystine exporter defective in cystinosis, is the founding member of a family of heptahelical membrane proteins related to bacteriorhodopsin and characterized by a duplicated motif termed the PQ loop. PQ-loop proteins are more frequent in eukaryotes than
Yujie Ma et al.
The Analyst, 138(18), 5338-5343 (2013-07-23)
A quantitative colorimetric sensing strategy utilizing cysteamine modified gold nanoparticles (CA-AuNPs) as reporters for Hg(2+) and melamine was demonstrated. Cysteamine is a cheap and commercially available aminothiol and is also the most important chelating ligand in coordination chemistry possessing the
Martine Besouw et al.
Drug discovery today, 18(15-16), 785-792 (2013-02-19)
Cysteamine is an amino thiol with the chemical formula HSCH2CH2NH2. Endogenously, cysteamine is derived from coenzyme A degradation, although its plasma concentrations are low. Most experience with cysteamine as a drug originates from the field of the orphan disease cystinosis
János Szolcsányi et al.
Current topics in medicinal chemistry, 11(17), 2253-2263 (2011-06-16)
Release of somatostatin into the circulation from the activated TRPV1-expressing nociceptors revealed by antidromic stimulation of dorsal roots in the rat pinpointed to a novel potential drug target on these nociceptors. The review summarizes the functional, biochemical and pharmacological evidence
Development of new treatments for Batten disease.
Sara E Mole
The Lancet. Neurology, 13(8), 749-751 (2014-07-07)
Sondra W Levin et al.
The Lancet. Neurology, 13(8), 777-787 (2014-07-07)
Infantile neuronal ceroid lipofuscinosis is a devastating neurodegenerative lysosomal storage disease caused by mutations in the gene (CLN1 or PPT1) encoding palmitoyl-protein thioesterase-1 (PPT1). We have previously reported that phosphocysteamine and N-acetylcysteine mediate ceroid depletion in cultured cells from patients
Iwan Zimmermann et al.
PLoS biology, 10(11), e1001429-e1001429 (2012-11-28)
The modulation of pentameric ligand-gated ion channels (pLGICs) by divalent cations is believed to play an important role in their regulation in a physiological context. Ions such as calcium or zinc influence the activity of pLGIC neurotransmitter receptors by binding
Rajib Ghosh et al.
Biomaterials, 34(3), 807-816 (2012-11-01)
Lack of affordable technologies for delivering microRNAs and siRNAs into cells on a large scale has hindered our efforts to rapidly parse through hundreds of dysregulated genes/microRNAs in order to identify drivers of complex diseases. The instability and polyanionic nature
Yanuar Dwi Putra Limasale et al.
International journal of pharmaceutics, 479(2), 364-373 (2015-01-18)
Cyclooxygenase-2 (COX-2) is highly expressed in many different cancers. Therefore, the inhibition of the COX-2 pathway by a selective COX-2 inhibitor, celecoxib (CLX), may be an alternative strategy for cancer prevention and therapy. Liposomal drug delivery systems can be used
Zhe Zhang et al.
The Analyst, 137(20), 4822-4828 (2012-09-04)
Sol-gel copolymer-templated mesoporous silica films with a thickness of 70 nm and interpore spacing of 4.34 nm were fabricated on gold layer covered glass substrates for application as a wavelength-interrogated surface plasmon resonance (SPR) sensor. The resonance wavelength (λ(R)) of
Giant cell transformation of podocytes: A unique histological feature associated with cystinosis.
Sharma A, Gupta R, Sethi SK, Bagga A, Dinda AK.
Colloids and Surfaces, B: Biointerfaces, 21, 123-125 (2011)
Judit García-Villoria et al.
Clinical biochemistry, 46(3), 271-274 (2012-10-20)
Cystinosis is an autosomal recessive genetic disorder due to mutations in CTNS gene, which causes a defect of cystinosin, impairing the transport of free cystine out of lysosomes and causing irreversible damage to various organs, particularly the kidney. The diagnosis
Reda Elshafey et al.
Biosensors & bioelectronics, 39(1), 220-225 (2012-08-18)
The detection of cancer biomarkers is as important tool for the diagnosis and prognosis of cancer such as brain cancer. Murine double minute 2 (MDM2) has been widely studied as prognostic marker for brain tumor. Here we describe development of
Ranjan Dohil et al.
Pediatric nephrology (Berlin, Germany), 28(3), 507-510 (2012-09-25)
Patients with nephropathic cystinosis are required to take 6-hourly immediate-release cysteamine (Cystagon®) to reduce disease progression. This arduous regimen affects quality of life, disrupts sleep, and may result in non-compliance with therapy. Enteric-coated cysteamine bitartrate (EC-cysteamine) was developed as a
Dong Zhang et al.
Inorganic chemistry, 52(4), 1676-1678 (2013-01-30)
The thiolato complex [platinum(II) (bipyridine)(N,S-aminoethanethiolate)](+)Ch(-) (1) undergoes sequential reactions with singlet oxygen to initially form the corresponding sulfenato complex [platinum(II) (bipyridine)(N,S(═O)-aminoethansulfenate)](+) (2) followed by a much slower reaction to the corresponding sulfinato complex. In contrast with many platinum dithiolato complexes
Paula Díez et al.
Analytical and bioanalytical chemistry, 405(11), 3773-3781 (2012-10-24)
Cysteamine core polyamidoamine G-4 dendron branched with β-cyclodextrins was chemisorbed on the surface of Au electrodes and further coated with Pt nanoparticles. Adamantane-modified glucose oxidase was subsequently immobilized on the nanostructured electrode surface by supramolecular association. This enzyme electrode was
Barbara Buchan et al.
Journal of pharmaceutical sciences, 101(10), 3729-3738 (2012-07-11)
Nephropathic cystinosis is a rare autosomal recessive disease characterised by raised lysosomal levels of cystine in the cells of all the organs. It is treated by the 6-h oral administration of the aminothiol, cysteamine, which has an offensive taste and
Keunhee Oh et al.
Clinical immunology (Orlando, Fla.), 145(2), 122-132 (2012-09-25)
The increased activity of transglutaminase 2 (TG2) in various inflammatory and fibrotic conditions results in the development of numerous disease processes. Experimental autoimmune encephalomyelitis (EAE), an animal model of multiple sclerosis, is an inflammatory and demyelinating disease of the central
Duk-Soo Kim et al.
BMB reports, 45(9), 532-537 (2012-09-27)
P18, a member of the INK4 family of cyclin-dependent kinase inhibitors, is a tumor suppressor protein and plays a key cell survival role in a variety of human cancers. Under pathophysiological conditions, the INK4 group proteins participate in novel biological
Anna Sankiewicz et al.
Analytical biochemistry, 469, 4-11 (2014-10-15)
We have developed a new method for highly selective determination of the ubiquitin carboxyl-terminal hydrolase L1 (UCH-L1) concentration using a surface plasmon resonance imaging (SPRI) technique and two different biosensors. UCH-L1 was captured from a solution by immobilized specific rabbit
Jiying Pei et al.
Analytica chimica acta, 757, 63-68 (2012-12-05)
High-quality cysteamine-coated CdTe quantum dots (CA-CdTe QDs) were successfully synthesized in aqueous phase by a facile one-pot method. Through hydroxylamine hydrochloride-promoted kinetic growth strategy, water-soluble CA-CdTe QDs could be obtained conveniently in a conical flask by a stepwise addition of
Sarah Unser et al.
The Analyst, 140(2), 590-599 (2014-11-27)
Development of improved glucose detection has vast significance in both clinical and point of care settings. Herein, we present a novel, label-free, enzyme-free, colorimetric method of glucose detection that relies on the reduction of a gold salt precursor facilitated by
Zhaolu Xie et al.
Biomaterials, 35(27), 7896-7908 (2014-06-16)
Mucus layer coating the vaginal epithelium represents a barrier for intravaginally delivered recombined adenoviral (rAd) vectors, but it could be overcome by proper polyethylene glycol (PEG) modification. Here we synthesized two cationic PEG derivatives, amino-(EO)n/(AGE)m-Cyss (APCs). The polymers contained neutral
Patricia Rodrigues Orsi et al.
Journal of ethnopharmacology, 143(1), 81-90 (2012-07-04)
Hymenaea stigonocarpa Mart. ex Hayne (Fabaceae) is a medicinal species commonly found in the Brazilian savannah. The stem bark of this medicinal plant, popularly known as "jatobá-do-cerrado", is widely used in tea form to treat gastric pain, ulcers, diarrhoea and
Electroactive SWNT/PEGDA hybrid hydrogel coating for bio-electrode interface.
He L, Lin D, Wang Y, Xiao Y, Che J.
Colloids and Surfaces, B: Biointerfaces, 87, 273-279 (2011)
Deni Rahmat et al.
Drug development and industrial pharmacy, 39(9), 1338-1345 (2012-08-21)
Within this study, the influence of particle size and zeta potential of hydroxyethyl cellulose-cysteamine particles on permeation enhancing properties was investigated. Particles were prepared by four different methods namely ionic gelation, spray drying, air jet milling and grinding. Particles prepared
Songjun Guo et al.
Isotopes in environmental and health studies, 48(4), 473-482 (2012-07-20)
δ(13)C values of gaseous acetaldehyde were measured by gas chromatograph-combustion-isotope ratio mass spectrometer (GC-C-IRMS) via sodium bisulfite (NaHSO(3)) adsorption and cysteamine derivatisation. Gaseous acetaldehyde was collected via NaHSO(3)-coated Sep-Pak(®) silica gel cartridge, then derivatised with cysteamine, and then the δ(13)C
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