Search

With a rising interest in smart windows and optical displays, the utilization of metal oxides (MOs) has garnered significant attention owing to their high active sites, flexibility, and tunable electronic and optical properties. Despite these advantages, achieving precise tuning of optical properties in MOs-based quantum dots and their mass production remains a challenge. In this study, we present an easily scalable approach to generate WO3 quantum dots with diverse sizes through sequential insertion/exfoliation processes in solvents with suitable surface tension. Additionally, we utilized the prepared WO3 quantum dots in the fabrication of luminescent transparent wood via an impregnation process. These quantum dots manifested three distinct emitting colors: red, green, and blue. Through characterizations of the structural and optical properties of the WO3 quantum dots, we verified that quantum dots with sizes around 30 nm, 50 nm, and 70 nm showcase a monoclinic crystal structure with oxygen-related defect sites. Notably, as the size of the WO3 quantum dots decreased, the maximum emitting peak underwent a blue shift, with peaks observed at 407 nm (blue), 493 nm (green), and 676 nm (red) under excitation by a He-Cd laser (310 nm), respectively. Transparent woods infused with various WO3 quantum dots exhibited luminescence in blue/white emitting colors. These results suggest substantial potential in diverse applications, such as building materials and optoelectronics. [ABSTRACT FROM AUTHOR]

The GGGGCC (G4C2) repeat expansion in a noncoding region of C9orf72 is the most common cause of sporadic and familial forms of amyotrophic lateral sclerosis and frontotemporal dementia. The basis for pathogenesis is unknown. To elucidate the consequences of G4C2 repeat expansion in a tractable genetic system, we generated transgenic fly lines expressing 8, 28 or 58 G4C2-repeat-containing transcripts that do not have a translation start site (AUG) but contain an open-reading frame for green fluorescent protein to detect repeat-associated non-AUG (RAN) translation. We show that these transgenic animals display dosage-dependent, repeat-length-dependent degeneration in neuronal tissues and RAN translation of dipeptide repeat (DPR) proteins, as observed in patients with C9orf72-related disease. This model was used in a large-scale, unbiased genetic screen, ultimately leading to the identification of 18 genetic modifiers that encode components of the nuclear pore complex (NPC), as well as the machinery that coordinates the export of nuclear RNA and the import of nuclear proteins. Consistent with these results, we found morphological abnormalities in the architecture of the nuclear envelope in cells expressing expanded G4C2 repeats in vitro and in vivo. Moreover, we identified a substantial defect in RNA export resulting in retention of RNA in the nuclei of Drosophila cells expressing expanded G4C2 repeats and also in mammalian cells, including aged induced pluripotent stem-cell-derived neurons from patients with C9orf72-related disease. These studies show that a primary consequence of G4C2 repeat expansion is the compromise of nucleocytoplasmic transport through the nuclear pore, revealing a novel mechanism of neurodegeneration.

Algorithms designed to identify canonical yeast prions predict that around 250 human proteins, including several RNA-binding proteins associated with neurodegenerative disease, harbour a distinctive prion-like domain (PrLD) enriched in uncharged polar amino acids and glycine. PrLDs in RNA-binding proteins are essential for the assembly of ribonucleoprotein granules. However, the interplay between human PrLD function and disease is not understood. Here we define pathogenic mutations in PrLDs of heterogeneous nuclear ribonucleoproteins (hnRNPs) A2B1 and A1 in families with inherited degeneration affecting muscle, brain, motor neuron and bone, and in one case of familial amyotrophic lateral sclerosis. Wild-type hnRNPA2 (the most abundant isoform of hnRNPA2B1) and hnRNPA1 show an intrinsic tendency to assemble into self-seeding fibrils, which is exacerbated by the disease mutations. Indeed, the pathogenic mutations strengthen a 'steric zipper' motif in the PrLD, which accelerates the formation of self-seeding fibrils that cross-seed polymerization of wild-type hnRNP. Notably, the disease mutations promote excess incorporation of hnRNPA2 and hnRNPA1 into stress granules and drive the formation of cytoplasmic inclusions in animal models that recapitulate the human pathology. Thus, dysregulated polymerization caused by a potent mutant steric zipper motif in a PrLD can initiate degenerative disease. Related proteins with PrLDs should therefore be considered candidates for initiating and perhaps propagating proteinopathies of muscle, brain, motor neuron and bone.

Spinal bulbar muscular atrophy (SBMA) is a motor neuron disease caused by toxic gain of function of the androgen receptor (AR). Previously, we found that co-regulator binding through the activation function-2 (AF2) domain of AR is essential for pathogenesis, suggesting that AF2 may be a potential drug target for selective modulation of toxic AR activity. We screened previously identified AF2 modulators for their ability to rescue toxicity in a Drosophila model of SBMA. We identified two compounds, tolfenamic acid (TA) and 1-[2-(4-methylphenoxy)ethyl]-2-[(2-phenoxyethyl)sulfanyl]-1H-benzimidazole (MEPB), as top candidates for rescuing lethality, locomotor function and neuromuscular junction defects in SBMA flies. Pharmacokinetic analyses in mice revealed a more favorable bioavailability and tissue retention of MEPB compared with TA in muscle, brain and spinal cord. In a preclinical trial in a new mouse model of SBMA, MEPB treatment yielded a dose-dependent rescue from loss of body weight, rotarod activity and grip strength. In addition, MEPB ameliorated neuronal loss, neurogenic atrophy and testicular atrophy, validating AF2 modulation as a potent androgen-sparing strategy for SBMA therapy., Author(s): Nisha M Badders [1]; Ane Korff [1, 2]; Helen C Miranda [3]; Pradeep K Vuppala [4]; Rebecca B Smith [1]; Brett J Winborn [1]; Emmanuelle R Quemin [1]; Bryce [...]

Objectives: In 2012, Liu et al. reported that miR-34 is an age-related miRNA regulating age-associated events and long-term brain integrity in Drosophila. They demonstrated that modulating miR-34 and its downstream target, Eip74EF, showed beneficial effects on an age-related disease using a Drosophila model of Spinocerebellar ataxia type 3 expressing SCA3trQ78. These results imply that miR-34 could be a general genetic modifier and therapeutic candidate for age-related diseases. Thus, the goal of this study was to examine the effect of miR-34 and Eip47EF on another age-related Drosophila disease model. Results: Using a Drosophila eye model expressing mutant Drosophila VCP (dVCP) that causes amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), or multisystem proteinopathy (MSP), we demonstrated that abnormal eye phenotypes generated by dVCPR152H were rescued by Eip74EF siRNA expression. Contrary to our expectations, miR-34 overexpression alone in the eyes with GMR-GAL4 resulted in complete lethality due to the leaky expression of GMR-GAL4 in other tissues. Interestingly, when miR-34 was co-expressed with dVCPR152H, a few survivors were produced; however, their eye degeneration was greatly exacerbated. Our data indicate that, while confirming that the downregulation of Eip74EF is beneficial to the dVCPR152HDrosophila eye model, the high expression level of miR-34 is actually toxic to the developing flies and the role of miR-34 in dVCPR152H-mediated pathogenesis is inconclusive in the GMR-GAL4 eye model. Identifying the transcriptional targets of Eip74EF might provide valuable insights into diseases caused by mutations in VCP such as ALS, FTD, and MSP. [ABSTRACT FROM AUTHOR]

In this paper, we propose new texture features, block difference of inverse probabilities (BDIP) and block variation of local correlation coefficients (BVLC), for content-based image retrieval and then present an image retrieval method based on the combination of BDIP and BVLC moments. BDIP uses local probabilities in image blocks to measure local brightness variations of an image well. BVLC uses variations of local correlation coefficients in image blocks to measure local texture smoothness of an image well. Experimental results show that the presented retrieval method yields about 12% better performance in precision vs. recall and about 0.13 in average normalized modified retrieval rank (ANMRR) than the method using wavelet moments. Index Terms--Block difference of inverse probabilities (BDIP), block variation of local correlation coefficients (BVLC), content-based image retrieval (CBIR), texture feature.

We propose a simple yet efficient method which reduces the blocking artifact in block-coded images by using a wavelet transform. An image is considered a set of one-dimensional signals, and so all processings including the wavelet transform are one-dimensionally executed. The artifact reduction operation is applied to only the neighborhood of each block boundary in the wavelet transform at the first and second scales. The key idea behind the method is to remove the blocking component which reveals stepwise discontinuities at block boundaries. Each block boundary is classified into one of shade region, smooth edge region, and step edge region. Threshold values for the classification are selected adaptively according to each coded image. The performance is evaluated for 512 x 512 images JPEG coded with 30:1 and 40:1 compression ratios. Experimental results show that the proposed method yields not only a PSNR improvement of about 0.69-1.06 dB, but also subjective quality nearly free of the blocking artifact and edge blur. Index Terms - Blocking artifact, JPEG, multiscale analysis, wavelet transform.

In this paper, a rate-distortion based image segmentation algorithm for segmentation-based coding is presented using a recursive merging with region adjacency graph (RAG). In the method, the dissimilarity between a pair of adjacent regions is represented as a Lagrangian cost function considered in a rate-distortion sense. Lagrange multiplier is estimated in each merging step, a pair of adjacent regions whose cost is minimal is searched, and then the pair of regions are merged into a new region. The merging step is recursively performed until some termination criterion is reached. The proposed method thus is suitable for region- or segmentation-based coding. Experimental results for 256 x 256 Lena show that segmentation-based coding using the proposed method yields PSNR improvement of about 3.5~4.5 dB, 1.8~2.0 dB, and 1.2~1.5 dB over mean-difference based method, distortion.based method, and JPEG, respectively. Index Terms--Image segmentation, RAG, rate-distortion, recursive merging, region-based coding.