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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
Bingjie Cai et al.
Biosensors & bioelectronics, 74, 329-334 (2015-07-15)
Early detection is proven to be the best chance for successful cancer treatment. MiRNAs, as ideal biomarkers, can identify cancer in the early stage. Therefore, development of highly sensitive and selective detection methods for miRNA is still anticipated. Here we
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
Pablo Liddle et al.
Molecular and cellular biochemistry, 473(1-2), 77-91 (2020-07-09)
In response to DNA double-strand breaks (DSB), histone H2AX is phosphorylated around the lesion by a feed forward signal amplification loop, originating γH2AX foci detectable by immunofluorescence and confocal microscopy as elliptical areas of uniform intensity. We exploited the significant
Janosch Peter Heller et al.
Methods (San Diego, Calif.), 174, 81-90 (2019-06-04)
Astroglia are vital facilitators of brain development, homeostasis, and metabolic support. In addition, they are also essential to the formation and regulation of synaptic circuits. Due to the extraordinary complex, nanoscopic morphology of astrocytes, the underlying cellular mechanisms have been
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
Ariane Roseblade et al.
Current cancer drug targets, 15(3), 205-214 (2015-02-26)
Microparticles (MPs) are released from most eukaryotic cells after the vesiculation of the plasma membrane and serve as vectors of long and short-range signaling. MPs derived from multidrug resistant (MDR) cancer cells carry molecular components of the donor cell such
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
Yan Yang et al.
Pharmacological research, 95-96, 71-81 (2015-04-04)
Curcumin (CUR) has been proven to be clinically effective in rheumatoid arthritis (RA) therapy, but its low oral bioavailability eclipses existent evidence that attempts to explain the underlying mechanism. Small intestine, the only organ exposed to a relatively high concentration
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
John E Dominy et al.
The Journal of biological chemistry, 282(35), 25189-25198 (2007-06-22)
There are only two known thiol dioxygenase activities in mammals, and they are ascribed to the enzymes cysteine dioxygenase (CDO) and cysteamine (2-aminoethanethiol) dioxygenase (ADO). Although many studies have been dedicated to CDO, resulting in the identification of its gene
B S Szwergold
Medical hypotheses, 66(4), 698-707 (2005-12-20)
Non-enzymatic glycation of proteins and some phospholipids is considered to be an important factor in the genesis of diabetic complications. While this process has been viewed traditionally as entirely non-enzymatic and unidirectional, the discovery of fructosamine-3-phosphate (FN3K) and identification of
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
Edward P Harvey et al.
Cell chemical biology, 27(6), 647-656 (2020-05-16)
The BCL-2 family is composed of anti- and pro-apoptotic members that respectively protect or disrupt mitochondrial integrity. Anti-apoptotic overexpression can promote oncogenesis by trapping the BCL-2 homology 3 (BH3) "killer domains" of pro-apoptotic proteins in a surface groove, blocking apoptosis.
Ekaterina Isaac et al.
Theriogenology, 161, 273-284 (2020-12-29)
The growth of viable cattle embryos in culture to stages beyond the hatching blastocyst is of interest to developmental biologists wishing to understand developmental events beyond the first lineage decision, as well as for commercial applications, because a lengthening of
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
Szilárd Szikora et al.
Bio-protocol, 10(12), e3654-e3654 (2021-03-05)
Sarcomeres are extremely highly ordered macromolecular assemblies where proper structural organization is an absolute prerequisite to the functionality of these contractile units. Despite the wealth of information collected, the exact spatial arrangement of many of the H-zone and Z-disk proteins
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
Ca Leisinger et al.
Animal reproduction science, 150(1-2), 70-75 (2014-09-28)
The present study utilized a 2×2×2 factorial design examining age (old vs. young), follicle size (≥2mm vs. <2mm) and media supplementation (with or without fetal bovine serum [FBS]) to determine factors that might affect in vitro maturation of alpaca oocytes.
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
Kessarin Ngamdee et al.
Analytica chimica acta, 898, 93-100 (2015-11-04)
A new chemical sensor based on the measuring of circular dichroism signal (CD) was fabricated from cysteamine capped cadmium sulfide quantum dots (Cys-CdS QDs). The chiral-thiol molecules, d-penicillamine (DPA) and l-penicillamine (LPA), were used to evaluate potentials of this sensor.
Jisoo Kim et al.
Micromachines, 9(10) (2018-11-15)
We present the plasmonic fiber based optical glucometer. A thin gold layer is coated on clad-free core of multimode optical fiber along 3 cm length to excite surface plasmons at 632.8 nm wavelength. Glucose oxidase is immobilized on the metal
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