Mutant dominant-negative causes protein aggregates degraded via ERAD and prevents normal rhodopsin from proper membrane trafficking.

Dominant mutations in the rhodopsin gene () contribute to 25% of autosomal dominant retinitis pigmentosa (adRP), characterized by photoreceptor loss and progressive blindness. One such mutation, carries a 3-bp deletion, resulting in the loss of one of two isoleucines at codons 255 and 256. Our investigation, using recombinant expression in HEK293 and COS-7 cells, revealed that , akin to the known adRP mutation , induces the formation of rhodopsin protein (RHO) aggregates at the perinuclear region.

Soft bioelectronics for diagnostic and therapeutic applications in neurological diseases.

Physical and chemical signals in the central nervous system yield crucial information that is clinically relevant under both physiological and pathological conditions. The emerging field of bioelectronics focuses on the monitoring and manipulation of neurophysiological signals with high spatiotemporal resolution and minimal invasiveness. Significant advances have been realized through innovations in materials and structural design, which have markedly enhanced mechanical and electrical properties, biocompatibility, and overall device performance.

A high spatial resolution dataset of China's biomass resource potential.

Assessing biomass resource potential is essential for China's ambitious goals of carbon neutrality, rural revitalization, and poverty eradication. To fill the data gap of high spatial resolution biomass resources in China, this study estimates the biomass resource potential for all types of lignocellulosic biomass feedstock at 1 km resolution in 2018, including 9 types of agricultural residues, 11 types of forestry residues, and 5 types of energy crops. By combining the statistical accounting method and the GIS-based method, this study develops a transparent and comprehensive assessment framework, which is in accordance with the principle of food security, forest land and pasture protection, and biodiversity protection.

Non-traditional cognitive brain network involvement in insulo-Sylvian gliomas: a case series study and clinical experience using Quicktome.

Background: Patients with insulo-Sylvian gliomas continue to present with severe morbidity in cognitive functions primarily due to neurosurgeons' lack of familiarity with non-traditional brain networks. We sought to identify the frequency of invasion and proximity of gliomas to portions of these networks.

Methods: We retrospectively analyzed data from 45 patients undergoing glioma surgery centered in the insular lobe.

Structural alterations of the salience network in patients with insular glioma.

Objective: The structural alteration that occurs within the salience network (SN) in patients with insular glioma is unclear. Therefore, we aimed to investigate the changes in the topological network and brain structure alterations within the SN in patients with insular glioma.

Methods: We enrolled 46 patients with left insular glioma, 39 patients with right insular glioma, and 21 demographically matched healthy controls (HCs).

In Situ Self-Assembly of Nanoscale Particles into Macroscale Ordered Monolayers with Enhanced Memory Performance.

In situ fabrication of macroscale ordered monolayers of nanoparticles (NPs) on targeted substrates is highly desirable for precision electronic and optical devices, while it remains a great challenge. In this study, a solution is provided to address this challenge by developing a colloidal ink formulation and employing the direct-ink-writing (DIW) technique, where on-demand delivery of ink at a targeted location and directional evaporation with controllable rate are leveraged to precisely guide the deposition of polystyrene-grafted gold NPs (Au@PS NPs) into a macroscale monolayer with an ordered Au NP array embedded in a PS thin film. A 2D steady-state diffusion-controlled evaporation model, which explains the parameter dependence of the experimental results and gives semiquantitative agreement with the experimental evaporation kinetics is proposed.

Alternative Pathway to Phase Down Coal Power and Achieve Negative Emission in China.

Although widely recognized as the key to climate goals, coal "phase down" has long been argued for its side effects on energy security and social development. Retrofitting coal power units with biomass and coal co-firing with a carbon capture and storage approach provides an alternative way to avoid these side effects and make deep carbon dioxide emission cuts or even achieve negative emission. However, there is a lack of clear answers to how much the maximum emission reduction potential this approach can unlock, which is the key information to promote this technology on a large scale.

Visible-Light Organophotoredox-Mediated [3 + 2] Cycloaddition of Arylcyclopropylamine with Structurally Diverse Olefins for the Construction of Cyclopentylamines and Spiro[4.] Skeletons.

We developed a visible-light-mediated [3 + 2] cycloaddition of arylcyclopropylamine with structurally diverse olefins using as a highly efficient organic photoredox catalyst. We first achieved the use of various alkyl-substituted alkenes in intermolecular [3 + 2] cycloadditions with cyclopropylamine. We also developed a general and efficient strategy for the construction of structurally diverse cyclopentane-based spiro[4.

Model Systems for Studies Into Retinal Neuroprotection.

Therapy development for neurodegenerative diseases of the retina constitutes a major unmet medical need, and this may be particularly relevant for inherited diseases of the retina, which are largely untreatable to this day. Therapy development necessitates appropriate models to improve the understanding of the underlying degenerative mechanisms, as well as for the testing and evaluation of novel treatment approaches. This review provides an overview of various model systems used to study retinal neuroprotection.

Large-area mid-infrared broadband absorbers based on spiral ITO resulting from the combination of two different broadening absorption methods.

A large-area mid-infrared broadband absorber is proposed in this paper. The absorber is a spiral ITO structure grown on a hexagonal lattice arrangement of silicon nanopillars by using a glancing angle deposition method. The experimental results show that when the heights of the silicon nanopillars are 1.

Pharmacological Inhibition of the VCP/Proteasome Axis Rescues Photoreceptor Degeneration in RHO Rat Retinal Explants.

Rhodopsin () misfolding mutations are a common cause of the blinding disease autosomal dominant retinitis pigmentosa (adRP). The most prevalent mutation, , results in its misfolding and retention in the endoplasmic reticulum (ER). Under homeostatic conditions, misfolded proteins are selectively identified, retained at the ER, and cleared via ER-associated degradation (ERAD).

An adaptive PID-type sliding mode learning compensation of torque ripple in PMSM position servo systems towards energy efficiency.

In this paper, for periodic motion tasks, combining adaptive PID-type sliding mode control (APIDSMC), model reference adaptive control (MRAC) and periodic adaptive learning control (PALC), a novel APIDSMC-PALC compensation approach towards energy efficiency is proposed to suppress the influence of torque ripple in permanent magnet synchronous motor (PMSM) servo systems. Using particle swarm optimization (PSO) algorithm, the equivalent control gain of sliding mode control is optimized to achieve energy efficiency during long-term operation. The objective of the proposed ripple compensation algorithm is to accurately approximate two dominant harmonic amplitudes in the torque ripple and generate an additional control effort for ripple compensation.

CT-Based Deep Learning Model for Invasiveness Classification and Micropapillary Pattern Prediction Within Lung Adenocarcinoma.

Identification of tumor invasiveness of pulmonary adenocarcinomas before surgery is one of the most important guides to surgical planning. Additionally, preoperative diagnosis of lung adenocarcinoma with micropapillary patterns is also critical for clinical decision making. We aimed to evaluate the accuracy of deep learning models on classifying invasiveness degree and attempted to predict the micropapillary pattern in lung adenocarcinoma.

Downregulation of Long Noncoding RNA MIAT in the Retina of Diabetic Rats with Tail-vein Injection of Human Umbilical-cord Mesenchymal Stem Cells.

Diabetic retinopathy (DR) is the common and important cause for visual impairment and blindness in working-aged people. Microangiopathy and inflammatory reactions are the key components of DR. Recently, long non-coding RNA myocardial infarction-associated transcript (MIAT) has emerged as a vital player in regulation for inflammatory processes and microvascular dysfunction.

A novel diagnostic method for pituitary adenoma based on magnetic resonance imaging using a convolutional neural network.

Purpose: This study was designed to develop a computer-aided diagnosis (CAD) system based on a convolutional neural network (CNN) to diagnose patients with pituitary tumors.

Methods: We included adult patients clinically diagnosed with pituitary adenoma (pituitary adenoma group), or adult individuals without pituitary adenoma (control group). After pre-processing, all the MRI data were randomly divided into training or testing datasets in a ratio of 8:2 to create or evaluate the CNN model.

L-shaped ITO structures fabricated by oblique angle deposition technique for mid-infrared circular dichroism.

This paper proposes a mid-infrared chiral structure, which consists of L-shaped indium tin oxide (ITO) films formed on self-assembled monolayer polystyrene microspheres in two orthogonal directions by oblique angle deposition technique. Experimental results demonstrate that the structure exhibit circular dichroism (CD) responses in the range of 2.5 - 4 µm.

Neuron-derived netrin-1 and netrin-4 proteins are additional effective targets in diabetic retinopathy beyond VEGF.

Vascular endothelial growth factor (VEGF) is the typical representative factor of diabetic retinopathy (DR) and is considered to be a key inducer of retinal vascular permeability in DR. Anti-VEGF has been widely used in clinical treatment but every patient is effective, therefore, it is necessary to find other effective factors that participate in the pathology of DR. We provide evidence from both human and animal experiments for the considerable roles of classical neuronal guidance factors, netrin-1 and netrin-4, in indicating and amending the pathology of DR.

Performance of an optical equalizer in a 10 G wavelength converting optical access network.

A centralized optical processing unit (COPU) that functions both as a wavelength converter (WC) and optical burst equaliser in a 10 Gb/s wavelength-converting optical access network is proposed and experimentally characterized. This COPU is designed to consolidate drifting wavelengths generated with an uncooled laser in the upstream direction into a stable wavelength channel for WDM backhaul transmission and to equalize the optical loud/soft burst power in order to relax the burst-mode receiver dynamic range requirement. The COPU consists of an optical power equaliser composed of two cascaded SOAs followed by a WC.