Intelligent Monitoring and Visualization System for High Building Nighttime Utilization Based on Image Processing.

The rise of complex high-rise buildings has made building management increasingly challenging, especially the nighttime supervision of university laboratories. Idle occupation increases the risk of accidents and undermines campus sustainability. Effective occupancy detection is essential for optimizing campus building safety and energy efficiency.

Elevated serum circulating cell-free mitochondrial DNA in amyotrophic lateral sclerosis.

Background And Purpose: The substantial role of inflammation in amyotrophic lateral sclerosis (ALS) is gaining support from recent research. Studies indicate that circulating cell-free mitochondrial DNA (ccf-mtDNA) can activate the immune system and is associated with neurodegenerative diseases. This research was designed to quantify ccf-mtDNA levels in the serum of ALS patients.

Investigation of the Effect of Compression Pressure in Contact OCT Imaging on the Measurement of Epidermis Thickness.

Optical coherence tomography (OCT) is a noninvasive 3D imaging technique that offers significant advantages over traditional microscopy and biopsy in measuring epidermal thickness (ET) when assessing skin conditions. However, OCT imagining is often required to be in a contact mode for mitigating the issues of subject movement and uneven skin topology. It is not known whether the contact would affect the ability of ET measurements.

Multiomics analysis reveals serine catabolism as a potential therapeutic target for MELAS.

Mitochondrial disease is a devastating genetic disorder, with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) and m.3243A>G being the most common phenotype and genotype, respectively. The treatment for MELAS patients is still less effective.

Cu(II) complex that synergistically potentiates cytotoxicity and an antitumor immune response by targeting cellular redox homeostasis.

Developing anticancer drugs with low side effects is an ongoing challenge. Immunogenic cell death (ICD) has received extensive attention as a potential synergistic modality for cancer immunotherapy. However, only a limited set of drugs or treatment modalities can trigger an ICD response and none of them have cytotoxic selectivity.

Quantifying Microvascular Structure in Healthy and Infarcted Rat Hearts Using Optical Coherence Tomography Angiography.

Myocardial infarction (MI) is a life-threatening medical emergency resulting in coronary microvascular dysregulation and heart muscle damage. One of the primary characteristics of MI is capillary loss, which plays a significant role in the progression of this cardiovascular condition. In this study, we utilized optical coherence tomography angiography (OCTA) to image coronary microcirculation in fixed rat hearts, aiming to analyze coronary microvascular impairment post-infarction.

A novel deep intronic variant introduce pseudoexon in Becker muscular dystrophy: A case report.

Most pathogenic variants are detectable and interpretable by standard genetic testing for dystrophinopthies. However, approximately 1∼3% of dystrophinopthies patients still do not have a detectable variant after standard genetic testing, most likely due to structural chromosome rearrangements and/or deep intronic pseudoexon-activating variants. Here, we report on a boy with a suspected diagnosis of Becker muscular dystrophy (BMD) who remained without a detectable variant after exonic DNA-based standard genetic testing.

Ion Pairing Enables Targeted Prodrug Activation via Red Light Photocatalysis: A Proof-of-Concept Study with Anticancer Gold Complexes.

Photocatalysis has found increasing applications in biological systems, for example, in localized prodrug activation; however, high-energy light is usually required without giving sufficient efficiency and target selectivity. In this work, we report that ion pairing between photocatalysts and prodrugs can significantly improve the photoactivation efficiency and enable tumor-targeted activation by red light. This is exemplified by a gold-based prodrug () functionalized with a morpholine moiety.

Clinical and genetic interpretation of uncertain DMD missense variants: evidence from mRNA and protein studies.

Background: Pathogenic missense variants in the dystrophin (DMD) gene are rarely reported in dystrophinopathies. Most DMD missense variants are of uncertain significance and their pathogenicity interpretation remains complicated. We aimed to investigate whether DMD missense variants would cause aberrant splicing and re-interpret their pathogenicity based on mRNA and protein studies.

Recent advances in hypoxia-activated compounds for cancer diagnosis and treatment.

Hypoxia, as a prevalent feature of solid tumors, is correlated with tumorigenesis, proliferation, and invasion, playing an important role in mediating the drug resistance and affecting the cancer treatment outcomes. Due to the distinct oxygen levels between tumor and normal tissues, hypoxia-targeted therapy has attracted significant attention. The hypoxia-activated compounds mainly depend on reducible organic groups including azo, nitro, N-oxides, quinones and azide as well as some redox-active metal complex that are selectively converted into active species by the increased reduction potential under tumor hypoxia.

A new pseudoexon activation due to ultrarare branch point formation in Duchenne muscular dystrophy.

Deep-intronic variants that create or enhance a splice site are increasingly reported as a significant cause of monogenic diseases. However, deep-intronic variants that activate pseudoexons by affecting a branch point are extremely rare in monogenic diseases. Here, we describe a novel deep-intronic DMD variant that created a branch point in a Duchenne muscular dystrophy (DMD) patient.

The clinical, myopathological, and genetic analysis of 155 Chinese mitochondrial ophthalmoplegia patients with mitochondrial DNA single large deletions.

Background: Progressive external ophthalmoplegia (PEO) is a common subtype of mitochondrial encephalomyopathy.

Objective: The study aimed to investigate the relationship between mitochondrial DNA (mtDNA) abnormalities, muscle pathology, and clinical manifestations in Chinese patients with single large-scale mtDNA deletion presenting with PEO.

Methods: This is a retrospective single-center study.

Cryptic exon activation caused by a novel deep-intronic splice-altering variant in Becker muscular dystrophy.

Background: An accurate genetic diagnosis of Becker muscular dystrophy (BMD) can be sometimes challenging due to deep intronic DMD variants. Here, we report on the genetic diagnosis of a BMD patient with a novel deep-intronic splice-altering variant in DMD.

Methods: The index case was a 3.

Clinical, pathological, and genetic characterization in a large Chinese cohort with female dystrophinopathy.

We aimed to investigate the clinical, pathological, and genetic characteristics of Chinese female dystrophinopathy and to identify possible correlations among them. One hundred forty genetically and/or pathologically confirmed female DMD variant carriers were enrolled, including 104 asymptomatic carriers and 36 symptomatic carriers. Twenty of 36 symptomatic and 16 of 104 asymptomatic carriers were sporadic with no family history.

Stacking thick perfusable human microvascular grafts enables dense vascularity and rapid integration into infarcted rat hearts.

Fabrication of large-scale engineered tissues requires extensive vascularization to support tissue survival and function. Here, we report a modular fabrication approach, by stacking of patterned collagen membranes, to generate thick (2 mm and beyond), large, three-dimensional, perfusable networks of endothelialized vasculature. In vitro, these perfusable vascular networks exhibit remodeling and evenly distributed perfusion among layers, while maintaining their patterned, open-lumen architecture.

Novel mutations in FLVCR1 cause tremors, sensory neuropathy with retinitis pigmentosa.

The mutations of the feline leukemia virus subgroup C receptor-related protein 1 (FLVCR1) cause ataxia with retinitis pigmentosa. Recent studies indicated a large variation in the phenotype of FLVCR1-associated diseases. In this report, we describe an adult male who manifested first with tremors in his third decade, followed by retinitis pigmentosa, sensory ataxia, and sensory neuropathy in his fourth decade.

Novel variants, muscle imaging, and myopathological changes in Chinese patients with VCP-related multisystem proteinopathy.

Objective: The objective of this research was to study the clinical features, genetic characteristics, muscle imaging, and muscle pathological changes of a cohort of Chinese patients with mutations in the valosin-containing protein (VCP) gene.

Methods: Nine patients from seven Chinese pedigrees were recruited. Variants were detected by next-generation sequencing and confirmed by Sanger sequencing.

Preparation of Carborane-Tailored Covalent Organic Frameworks by a Postsynthetic Modification Strategy as a Barrier to Polysulfide in Lithium-Sulfur Batteries.

Lithium-sulfur batteries (LSBs) have attracted much attention due to their high energy density and theoretical specific capacity. However, the "shuttle effect" of polysulfides limits their application. Herein, we propose a postsynthetic modification (PSM) strategy to synthesize a fibrous carborane-tailored covalent organic framework (PMCB-COF).

Pathogenic PSAT1 Variants and Autosomal Recessive Axonal Charcot-Marie-Tooth Disease With Ichthyosis.

Background: Biallelic pathogenic phosphoserine aminotransferase 1 (PSAT1) variants generally cause a severe phenotype predominantly involving the central nervous system. Here, for the first time, we report two patients harboring pathogenic PSAT1 variants only manifested as polyneuropathy and ichthyosis.

Methods: Two patients from unrelated families presenting with polyneuropathy and ichthyosis were enrolled.

Comparison of cytokine/chemokine profiles between dermatomyositis and anti-synthetase syndrome.

Objectives: Dermatomyositis (DM) and anti-synthetase syndrome (ASS) are autoimmune diseases with multisystem involvement. Despite sharing some clinical and myopathological features, these are two diseases with different pathogeneses and prognoses. We aimed to clarify and compare cytokine/chemokine profiles in both disorders, which may help in the differential diagnosis.

An in-frame pseudoexon activation caused by a novel deep-intronic variant in the dysferlin gene.

The precise detection and interpretation of pathogenic DYSF variants are sometimes challenging, largely due to rare deep-intronic splice-altering variants. Here, we report on the genetic diagnosis of a male patient with dysferlinopathy. He remained genetically unsolved after routine exonic detection approaches that only detected a novel heterozygous frameshift variant (c.