From onset to blindness: a comprehensive analysis of -associated X-linked retinopathy in a large cohort in China.

Background: Variants in the are the leading cause of X-linked retinopathies (XLRPs). Further in-depth investigation is needed to understand the natural history.

Methods: Review of all case records, molecular genetic testing results, best-corrected visual acuity (BCVA), retinal imaging data (including fundus autofluorescence imaging and optical coherence tomography (OCT)), static visual field (VF) assessments and full-field electroretinogram.

Metabolic self-feeding in HBV-associated hepatocarcinoma centered on feedback between circulation lipids and the cellular MAPK/mTOR axis.

Introduction: Hepatitis B Virus (HBV) is widely recognized as a "metabolic virus" that disrupts hepatic metabolic homeostasis, rendering it one of the foremost risk factors for hepatocellular carcinoma (HCC). Except for antiviral therapy, the fundamental principles underlying HBV and HBV HCC have remained unchanged, limiting HCC treatment options.

Objectives: In this study, we aim to identify the distinctive metabolic profile of HBV-associated HCC, with the promise of identifying novel metabolic targets that confer survival advantages and ultimately impede cancer progression.

Whole genome sequencing enables new genetic diagnosis for inherited retinal diseases by identifying pathogenic variants.

Inherited retinal diseases (IRDs) are a group of common primary retinal degenerative disorders. Conventional genetic testing strategies, such as panel-based sequencing and whole exome sequencing (WES), can only elucidate the genetic etiology in approximately 60% of IRD patients. Studies have suggested that unsolved IRD cases could be attributed to previously undetected structural variants (SVs) and intronic variants in IRD-related genes.

Design of Planar Differential Microphone Array Beampatterns with Controllable Mainlobe Beamwidth and Sidelobe Level.

The differential microphone array, or differential beamformer, has attracted much attention for its frequency-invariant beampattern, high directivity factor and compact size. In this work, the design of differential beamformers with small inter-element spacing planar microphone arrays is concerned. In order to exactly control the main lobe beamwidth and sidelobe level and obtain minimum main lobe beamwidth with a given sidelobe level, we design the desired beampattern by applying the Chebyshev polynomials at first, via exploiting the structure of the frequency-independent beampattern of a theoretical th-order differential beamformer.

Bufalin Inhibits Tumorigenesis and SREBP-1-Mediated Lipogenesis in Hepatocellular Carcinoma via Modulating the ATP1A1/CA2 Axis.

Altered lipid metabolism is a hallmark of hepatocellular carcinoma (HCC), a common malignancy with a dismal prognosis against which there is a lack of effective therapeutic strategies. Bufalin, a classical Na[Formula: see text]-K[Formula: see text]-ATPase (NKA) inhibitor, shows a potent antitumor effect against HCC. However, the role of bufalin in regulating lipid metabolism-related pathways of HCC remains unclear.

Flower-like Composite Material Delivery of Co-Packaged Lenvatinib and Bufalin Prevents the Migration and Invasion of Cholangiocarcinoma.

The co-delivery of multiple drugs using nanocarriers has been recognized as a promising strategy for cancer treatment to enhance therapeutic efficacy. In this study, a monodisperse mesoporous silica nanoparticle (mSiO) is prepared and functionalized into high-efficiency loaded Lenvatinib and Bufalin for targeted delivery to Cholangiocarcinoma (CCA). mSiO was synthesized on solid silica nanoparticles by oil-water interface method, and highly monodisperse mSiO with uniform morphology was obtained.

Identification and Validation of a Tumor Microenvironment-Related Gene Signature in Hepatocellular Carcinoma Prognosis.

Hepatocellular carcinoma (HCC) is a typical inflammatory-related malignant tumor with complex immune tolerance microenvironment and poor prognosis. In this study, we aimed to construct a novel immune-related gene signature for the prognosis of HCC patients, exploring tumor microenvironment (TME) cell infiltration characterization and potential mechanisms. A total of 364 HCC samples with follow-up information in the TCGA-LIHC dataset were analyzed for the training of the prognostic signature.

Botanical Drug Puerarin Promotes Neuronal Survival and Neurite Outgrowth against MPTP/MPP-Induced Toxicity via Progesterone Receptor Signaling.

Background: Progesterone receptor (PR) modulates neuroprotective and regenerative responses in Parkinson's disease and related neurological diseases.

Objectives: The present study was designed to determine whether botanical drug puerarin could exhibit neuroprotective and neurorestorative activities via PR signaling.

Methods: The neuroprotective and neurotrophic activities of puerarin were investigated in MPTP-lesioned mice and MPP-challenged primary rat midbrain neurons.

Model-based distributed node clustering and multi-speaker speech presence probability estimation in wireless acoustic sensor networks.

The knowledge of speech presence probability (SPP) plays an essential role in noise estimation and speech enhancement. Single channel SPP estimation and centralized multi-channel SPP estimation have been well studied. However, how to estimate SPP in wireless acoustic sensor networks (WASNs) remains a great challenge and few efforts can be found in this topic, particularly for WASN applications with multiple speakers.

N-Propargyl caffeate amide (PACA) prevents cardiac fibrosis in experimental myocardial infarction by promoting pro-resolving macrophage polarization.

Macrophages control the initiation and resolution of cardiac fibrosis in post-infarction cardiac remodeling. The aim of the present study was to investigate whether N-propargyl caffeate amide (PACA) could suppress myocardial fibrosis via regulating macrophage polarization. By using rat model of isoproterenol-induced myocardial fibrosis, we discovered that PACA could reduce cardiac fibrosis in a dose-dependent manner.

Interplay between Exosomes and Autophagy in Cardiovascular Diseases: Novel Promising Target for Diagnostic and Therapeutic Application.

Exosome, is identified as a nature nanocarrier and intercellular messenger that regulates cell to cell communication. Autophagy is critical in maintenance of protein homeostasis by degradation of damaged proteins and organelles. Autophagy and exosomes take pivotal roles in cellular homeostasis and cardiovascular disease.

Electro-Acupuncture Ameliorated MPTP-Induced Parkinsonism in Mice via TrkB Neurotrophic Signaling.

Neurotrophins, such as brain-derived neurotrophic factor (BDNF), have shown promise as neuroprotective agents, indicating their potential in therapeutic strategies for neurodegenerative disease. However, the inherent bioactivity and pharmaceutical limitations of BDNF compromise its clinical efficacy. Research has documented the beneficial effects of electroacupuncture (EA) against neurodegeneration, possibly by BDNF-mediated mechanisms.

Weight reduction and cardiovascular benefits: Protocol for a systematic review and meta-analysis.

Background: There is widespread obesity paradox in cardiovascular diseases, the cardiovascular influence from weight management remains controversial. Moreover, previous publications indicating that different weight reduction extent might lead to various results. Thus, it is of importance to reassess the cardiovascular benefits of weight management strategies.

A mechanistic overview of herbal medicine and botanical compounds to target transcriptional factors in Breast cancer.

The abnormalities of transcription factors, such as NF-κB, STAT, estrogen receptor, play a critical role in the initiation and progression of breast cancer. Due to the limitation of current treatment, transcription factors could be promising therapeutic targets, which have received close attention. In this review, we introduced herbal medicines, as well as botanical compounds that had been verified with anti-tumor properties via regulating transcription factors.

Roles and Mechanisms of Herbal Medicine for Diabetic Cardiomyopathy: Current Status and Perspective.

Diabetic cardiomyopathy is one of the major complications among patients with diabetes mellitus. Diabetic cardiomyopathy (DCM) is featured by left ventricular hypertrophy, myocardial fibrosis, and damaged left ventricular systolic and diastolic functions. The pathophysiological mechanisms include metabolic-altered substrate metabolism, dysfunction of microvascular, renin-angiotensin-aldosterone system (RAAS) activation, oxidative stress, cardiomyocyte apoptosis, mitochondrial dysfunction, and impaired Ca handling.

Meta-Analysis of the Effect of Obstructive Sleep Apnea on Cardiovascular Events After Percutaneous Coronary Intervention.

Increasing evidence proved that obstructive sleep apnea (OSA) is associated with a variety of diseases, especially cardiovascular diseases. This study systematically reviewed the existing publications to assess the influence of OSA on cardiovascular survival among patients who underwent percutaneous coronary intervention. Eleven eligible publications, including 3,008 participants, were finally evaluated.

Mechanisms and Clinical Application of Tetramethylpyrazine (an Interesting Natural Compound Isolated from Ligusticum Wallichii): Current Status and Perspective.

Tetramethylpyrazine, a natural compound from (), has been extensively used in China for cardiovascular and cerebrovascular diseases for about 40 years. Because of its effectiveness in multisystems, especially in cardiovascular, its pharmacological action, clinical application, and the structural modification have attracted broad attention. In this paper its mechanisms of action, the clinical status, and synthetic derivatives will be reviewed briefly.

Chinese herbal medicine bushen qinggan formula for blood pressure variability and endothelial injury in hypertensive patients: a randomized controlled pilot clinical trial.

Background. Blood pressure variability (BPV) independent of average blood pressure is related to cardiovascular damage. Meanwhile, BPV is also associated with measures of endothelial injury.