Wilson Disease in Infancy through Adolescence

Kerkar, Nanda; Roberts, Eve A.

doi:10.1016/b978-0-12-810532-0.00017-3

Cited by 4 publications

(3 citation statements)

References 101 publications

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“…However, prolonged pesticide usage has caused toxic components to accumulate in the environment, development of copper-resistant pathogens, and it negatively impact human health through the food chain ( Adrees et al., 2015 ; Costa et al., 2018 ; Lamichhane et al., 2018 ; Kering et al., 2019 ). Human medical conditions related to copper toxicity, include gastrointestinal, liver, and neurological illnesses (Alzheimer’s disease) ( Roychoudhury et al., 2016 ; Navarro and Schneuwly, 2017 ; Kerkar and Roberts, 2018 ; Kering et al., 2019 ). Resistance to copper-based fungicides is also a challenge in controlling of the pathogen, and copper resistance has been reported in numerous plant diseases, including Pseudomonas and Xanthomonas spp.…”

Section: Introductionmentioning

confidence: 99%

Phage combination alleviates bacterial leaf blight of rice (Oryza sativa L.)

Jiang

Xuefang

et al. 2023

Front. Plant Sci.

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Rice bacterial leaf blight (BLB) is the most destructive bacterial diseases caused by Xanthomonas oryzae pv. oryzae (Xoo). Phages have been proposed as a green and efficient strategy to kill bacterial pathogens in crops, however, the mechanism of action of phages in the control of phyllosphere bacterial diseases remain unclear. Here, the glasshouse pot experiment results showed that phage combination could reduce the disease index by up to 64.3%. High-throughput sequencing technology was used to analyze the characteristics of phyllosphere microbiome changes and the results showed that phage combinations restored the impact of pathogen invasion on phyllosphere communities to a certain extent, and increased the diversity of bacterial communities. In addition, the phage combination reduced the relative abundance of epiphytic and endophytic Xoo by 58.9% and 33.9%, respectively. In particular, Sphingomonas and Stenotrophomonas were more abundant. According to structural equation modeling, phage combination directly and indirectly affected the disease index by affecting pathogen Xoo biomass and phage resistance. In summary, phage combination could better decrease the disease index. These findings provide new insights into phage biological control of phyllosphere bacterial diseases, theoretical data support, and new ideas for agricultural green prevention and control of phyllosphere diseases.

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Section: Introductionmentioning

confidence: 99%

Phage combination alleviates bacterial leaf blight of rice (Oryza sativa L.)

Jiang

Xuefang

et al. 2023

Front. Plant Sci.

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“…d -penicillamine is one of such polymers used as a template for synthesizing metallic nanoclusters. d -penicillamine, a copper chelator, is an FDA-approved drug used to treat Wilson’s disease, kidney stone, rheumatoid arthritis, and various heavy metal poisonings . A combination of d -penicillamine and cupric sulfate produced dose-dependent reduction in cell viability in MCF-7 cells by producing hydrogen peroxide (H 2 O 2 ) .…”

Section: Introductionmentioning

confidence: 99%

Transferrin Coated d-penicillamine–Au-Cu Nanocluster PLGA Nanocomposite Reverses Hypoxia-Induced EMT and MDR of Triple-Negative Breast Cancers

Shome

Ghosh

2021

ACS Appl. Bio Mater.

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Triple-negative breast cancer (TNBC), the most aggressive subtype of breast cancer, lacks effective targeted therapies due to negative expression of the targetable bioreceptors. Additionally, hypoxic condition in solid tumors contributes to the epithelial to mesenchymal transition (EMT), which aggravates cancer progression, multidrug resistance (MDR), migration, and stemness of the TNBC. A therapeutic module has been established in this regard by coating PLGA nanoparticle with D-penicillamine templated Au−Cu bimetallic nanoclusters. Further, the resultant nanomaterials were coated with recombinant transferrin protein to specifically target transferrin receptor overexpressing TNBC. The synthesized nanocomposites showed strong orange emission band at 630 nm with fluorescence quantum yield of 2%, rendering it suitable for theranostic applications. Experimental results demonstrated efficient cellular internalization and significant innate anti-cell proliferative potential of the nanocomposites. The fabricated nanocomposites were also able to induce cell death in spheroids, which was confirmed by live/dead dual staining results. Furthermore, when EMT-induced TNBC cells were treated with nanocomposites, they generated reactive oxygen species (ROS), depolarized the mitochondrial membrane potential, and induced apoptosis. Gene expression by real-time PCR indicated that treatment of EMT-induced TNBC cells with nanocomposites facilitated mesenchymal to epithelial transition (MET). In MDA-MB-468 cells, treatment with nanocomposites resulted in a 1.35-fold rise in E-cadherin an epithelial marker and a 1.36-fold decrease in vimentin a mesenchymal marker. Similarly, 2.87-fold and 1.76-fold decrease in stemness markers ALDH1A3 and EpCAM were observed in MDA-MB-231. Furthermore, 4.63-fold decrease in expression of ABCC1, a prominent contributor of MDR, was observed in MDA-MB-231. Protein expression studies revealed that nanocomposites reduced p-STAT-3 by 1.61-fold in MDA-MB-231 and by 7.8-fold in MDA-MB-468. Importantly, nanocomposites downregulated the expression of β-catenin by 3-fold in MDA-MB-231 and by 3.11-fold in MDA-MB-468. Downregulation of EMT with concomitant alteration of STAT-3 and β-catenin signaling pathways led to reduced migration ability of the TNBC cells.

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“…With the help of mutation analysis for diagnosis the natural history of WD is changed. More and more young presymptomatic WD patients have been diagnosed before adolescence and treatment can be started early with the potential of preventing significant organ damage and hopefully attaining uneventful life expectancy similar to healthy individuals (6–9). Early and safe treatment of these presymptomatic WD with oral zinc therapy is effective in reducing dietary copper absorption and thus body copper load (6–9).…”

Section: Introductionmentioning

confidence: 99%

Reference intervals of spot urine copper excretion in preschool children and potential application in pre-symptomatic screening of Wilson’s disease

Tang

Hui

Huang

et al. 2019

Preprint

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43Background: 44 With spot urine collected from a large control sample of preschool children (aged 3-7 years), 45 reference range of spot urine copper excretion indexes and their biological variation were defined. 47Methods: 48In order to investigate their test performance in screening of Wilson disease in this age group, 49 multiple spot urine samples from 6 WD patients diagnosed at presymptomatic stage were analysed. 50 Cut-off values for spot urine copper concentration, copper to creatinine ratio and copper to 51 osmolality ratio at 0.5 µmol/L, 0.1 µmol/mmol and 0.00085 µmol/mOsmol (32 µg/L, 56 µg/g 52 creatinine and 0.054 µg/mOsmol, respectively, in conventional units) have potential application in 53 differentiation of WD patients. 54 55 Results: 56 The data provides a new insight that the inter-individual variation of spot urine copper indexes 57 (CVg) were moderate with figures around 60% which was similar to other clinically useful urine 58 tests, such as urine albumin excretion ratio. Spot urine copper excretion strongly correlated with 59both urine creatinine and osmolality. And more than 95% of data points in health preschool children 60 fell within prediction regions by linear regression suggesting a good utility of normalisation by these 61 2 analytes. Receiver operator curve (ROC) showed that copper to osmolality ratio was the best 62 index with an area under curve (AUC) greater than 0.98. 63 64 109 important in the interpretation of all laboratory test results. Although large scale programs like 110 CALIPER have been set up to better define RI in the paediatric age group, they are confined to 111 analytes in serum or blood samples (12). It is reasonable as they are more commonly encountered in 112 routine laboratories.113 114 157 for controls. In addition, the prediction region covering the predicted distribution of 95% control 158 data points were shown (blue rectangles in scatter plots, e.g. figure 2). Difference in spot urine 159 indexes are compared between WD and control by non-parametric group-wise statistics (Wilcoxon 160 test). Statistical significance was defined by type I error of 0.05.161 162

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Wilson Disease in Infancy through Adolescence

Cited by 4 publications

References 101 publications

Phage combination alleviates bacterial leaf blight of rice (Oryza sativa L.)

Phage combination alleviates bacterial leaf blight of rice (Oryza sativa L.)

Transferrin Coated d-penicillamine–Au-Cu Nanocluster PLGA Nanocomposite Reverses Hypoxia-Induced EMT and MDR of Triple-Negative Breast Cancers

Reference intervals of spot urine copper excretion in preschool children and potential application in pre-symptomatic screening of Wilson’s disease

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