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Pavel Lochman et al.
Electrophoresis, 24(7-8), 1200-1207 (2003-04-23)
Increased interest in the analysis of aminothiols in body fluids during the last years results in a request for high-throughput analytical methods for their determination. We report here a novel, high-throughput method for the determination of total concentrations of biogenous
Magdalena Ganz et al.
PloS one, 14(8), e0213130-e0213130 (2019-08-14)
DNA replication stress is a major source of genomic instability and is closely linked to tumor formation and progression. Poly(ADP-ribose)polymerases1/2 (PARP1/2) enzymes are activated in response to replication stress resulting in poly(ADP-ribose) (PAR) synthesis. PARylation plays an important role in
Sarada Viswanathan et al.
Nature methods, 12(6), 568-576 (2015-04-29)
We describe an engineered family of highly antigenic molecules based on GFP-like fluorescent proteins. These molecules contain numerous copies of peptide epitopes and simultaneously bind IgG antibodies at each location. These 'spaghetti monster' fluorescent proteins (smFPs) distributed well in neurons
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 F Pereira et al.
Zygote (Cambridge, England), 23(4), 485-493 (2014-04-17)
In vitro embryo production methods induce DNA damage in the embryos. In response to these injuries, histone H2AX is phosphorylated (γH2AX) and forms foci at the sites of DNA breaks to recruit repair proteins. In this work, we quantified the
The Hai Tran et al.
Advanced materials (Deerfield Beach, Fla.), 27(25), 3825-3830 (2015-05-27)
A novel bilayer coating system for autonomous corrosion-triggered self-healing is demonstrated. The storage of the encapsulated monomer and the catalyst is separated in two different layers. The encapsulated catalyst is stored inside a metallic coating, which ensures its activity even
Jake F Watson et al.
Nature communications, 12(1), 5083-5083 (2021-08-25)
AMPA receptor (AMPAR) abundance and positioning at excitatory synapses regulates the strength of transmission. Changes in AMPAR localisation can enact synaptic plasticity, allowing long-term information storage, and is therefore tightly controlled. Multiple mechanisms regulating AMPAR synaptic anchoring have been described
Mini Mol Menamparambath et al.
Scientific reports, 5, 16371-16371 (2015-11-18)
Silver nanowires have attracted much attention for use in flexible transparent conductive films (TCFs) due to their low sheet resistance and flexibility. However, the haze was too high for replacing indium-tin-oxide in high-quality display devices. Herein, we report flexible TCFs
Joyce M Geelen et al.
Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association, 17(10), 1766-1770 (2002-09-25)
Cystinosis is a rare autosomal recessive disease, caused by intracellular cystine accumulation due to a defect in the lysosomal cystine carrier. Treatment with cysteamine favours the transport of cystine out of the lysosomes, diminishes organ damage, and postpones the progression
Giorgio Mattei et al.
PloS one, 12(3), e0173206-e0173206 (2017-03-08)
Liver organoids (LOs) are of interest in tissue replacement, hepatotoxicity and pathophysiological studies. However, it is still unclear what triggers LO self-assembly and what the optimal environment is for their culture. Hypothesizing that LO formation occurs as a result of
Yasuhiro Fujiwara et al.
PLoS genetics, 16(9), e1009048-e1009048 (2020-09-16)
During meiotic prophase, sister chromatids are organized into axial element (AE), which underlies the structural framework for the meiotic events such as meiotic recombination and homolog synapsis. HORMA domain-containing proteins (HORMADs) localize along AE and play critical roles in the
Sang-Hun Lee et al.
The Journal of neuroscience : the official journal of the Society for Neuroscience, 35(27), 10039-10057 (2015-07-15)
Persistent CB1 cannabinoid receptor activity limits neurotransmitter release at various synapses throughout the brain. However, it is not fully understood how constitutively active CB1 receptors, tonic endocannabinoid signaling, and its regulation by multiple serine hydrolases contribute to the synapse-specific calibration
Alireza Molazemhosseini et al.
ACS applied electronic materials, 3(7), 3106-3113 (2021-09-07)
Biosensors are expected to revolutionize disease management through provision of low-cost diagnostic platforms for molecular and pathogenic detection with high sensitivity and short response time. In this context, there has been an ever-increasing interest in using electrolyte-gated field-effect transistors (EG-FETs)
Barbara C Sonies et al.
Medicine, 84(3), 137-146 (2005-05-10)
Nephropathic cystinosis is a rare, autosomal recessive lysosomal storage disorder caused by mutations in the CTNS gene that codes for a cystine transporter in the lysosomal membrane. Affected patients store 50-100 times the normal amounts of cystine in their cells
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
Carly G K Ziegler et al.
Cell reports, 28(4), 923-937 (2019-07-25)
In cancer biology, the functional interpretation of genomic alterations is critical to achieve the promise of genomic profiling in the clinic. For chronic lymphocytic leukemia (CLL), a heterogeneous disease of B-lymphocytes maturing under constitutive B cell receptor (BCR) stimulation, the
Gundula Min-Oo et al.
Mammalian genome : official journal of the International Mammalian Genome Society, 22(7-8), 486-494 (2011-03-26)
Malaria continues to be a serious threat to global health. The malaria problem is compounded by the absence of an efficacious vaccine and widespread drug resistance in the Plasmodium malarial parasite. The host factors and parasite virulence determinants that regulate
Tao Chen et al.
Journal of pharmaceutical and biomedical analysis, 117, 304-310 (2015-09-26)
Antibody-drug conjugates (ADCs) offer new therapeutic options for advanced cancer patients through precision killing with fewer side effects. The stability and efficacy of ADCs are closely related, emphasizing the urgency and importance of gaining a comprehensive understanding of ADC stability.
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
Daniel N Grant et al.
Journal of biomedical materials research. Part B, Applied biomaterials, 103(8), 1532-1540 (2014-12-04)
Extremely low frequency electromagnetic fields (ELF-EMFs) can induce beneficial effects including enhanced protein synthesis and cell proliferation on healing bone and skin wounds. This study investigated the effects of ELF-EMFs on acellular tissue constructs with and without gold nanoparticles (AuNPs)
Development of new treatments for Batten disease.
Sara E Mole
The Lancet. Neurology, 13(8), 749-751 (2014-07-07)
Atsushi Shoji et al.
Journal of pharmaceutical and biomedical analysis, 95, 47-53 (2014-03-19)
Evaluation of cathepsin B activities for degrading collagen IV and heat-denatured collagen IV (gelatin) were performed by surface plasmon resonance (SPR) and circular dichroism (CD) measurements. The optimal pH of cathepsin B activity for degrading each substrate was around 4.0.
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
Martin Lehmann et al.
Science advances, 5(11), eaax5775-eaax5775 (2019-12-07)
Clathrin-mediated endocytosis, an essential process for plasma membrane homeostasis and cell signaling, is characterized by stunning heterogeneity in the size and lifetime of clathrin-coated endocytic pits (CCPs). If and how CCP growth and lifetime are coupled and how this relates
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
Hye-Eun Lee et al.
ACS nano, 9(8), 8384-8393 (2015-07-15)
A concave rhombic dodecahedron (RD) gold nanoparticle was synthesized by adding 4-aminothiophenol (4-ATP) during growth from seeds. This shape is enclosed by stabilized facets of various high-indexes, such as (331), (221), and (553). Because it is driven thermodynamically and stabilized
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
S Orloff et al.
Pediatric research, 15(7), 1063-1067 (1981-07-01)
Cysteamine is the most effective agent known for the reduction of the elevated cystine content of cells from patients with cystinosis. A defect in endogenous cysteamine generation could account for many of the metabolic features of this disorder. To test
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