[Clinical efficacy of nusinersen sodium in the treatment of children with spinal muscular atrophy].

Objectives: To investigate the efficacy and safety of nusinersen sodium in the treatment of children with spinal muscular atrophy (SMA).

Methods: A retrospective analysis was conducted on the clinical data of 50 children with 5q SMA who received nusinersen sodium treatment and multidisciplinary treatment management in Shanxi Children's Hospital from February 2022 to February 2024.

Results: Compared with the baseline data, 67% (8/12), 74% (35/47), and 74% (35/47) of the SMA children had a clinically significant improvement in the scores of Philadelphia Infant Test of Neuromuscular Disorders, Hammersmith Functional Motor Scale Expanded, and Revised Upper Limb Module, respectively, and the distance of 6-minute walking test increased from 207.

CD160 Signaling Is Essential for CD8+ T Cell Memory Formation via Upregulation of 4-1BB.

A better understanding of the regulatory mechanisms governing the development of memory CD8+ T cells could provide instructive insights into vaccination strategies and T cell-based immunotherapies. In this article, we showed that CD160 surface protein is required for CD8+ T cell memory formation. In the response to acute lymphocytic choriomeningitis virus infection in a mouse model, CD160 ablation resulted in the failure of the development of all three memory CD8+ T cell subsets (central, effective, and tissue-resident memory), concomitant with a skewed differentiation into short-lived effector T cells.

A LY6E-PHB1-TRIM21 assembly degrades CD14 protein to mitigate LPS-induced inflammatory response.

A major theme of host against invading pathogens lies in multiple regulatory nodes that ensure sufficient signals for protection while avoiding excessive signals toward over-inflammation. The TLR4/MD-2/CD14 complex receptor-mediated response to bacterial lipopolysaccharide (LPS) represents a paradigm for understanding the proper control of anti-pathogen innate immunity. In this study, we studied the mechanism by which the glycosylphosphatidylinositol (GPI)-linked LY6E protein constrains LPS response via downregulating CD14.

Granulocytic myeloid-derived suppressor cells increase infection risk the IDO/IL-10 pathway in patients with hepatitis B virus-related liver failure.

Hepatitis B virus-related acute-on-chronic liver failure (HBV-ACLF) results in high susceptibility to infection. Although granulocytic myeloid-derived suppressor cells (gMDSC) are elevated in patients with HBV-ACLF, their role in HBV-ACLF pathogenesis is unknown. To elucidate the mechanism of gMDSC expansion and susceptibility to infection in HBV-ACLF patients, we analyzed the proportion of gMDSC in the peripheral blood and organ tissues of patients with HBV-ACLF and an ACLF mouse model established by continuous injection (eight times) of Concanavalin by flow cytometry and immunohistochemistry.

Improving the expansion of human tumor-reactive CD8 + T cells by targeting toll-like receptors.

Toll-like receptors (TLRs) are important pattern recognition receptor(s) known to mediate the sensing of invading pathogens and subsequent immune responses. In this study, we investigate whether TLRs could be explored for the preparation of human CD8 T cell products used in adoptive cell therapy (ACT). Following characterization of TLRs expression on human CD8 T cells, we screened TLR-specific agonists for their ability to act in concert with anti-CD3 to stimulate the proliferation of these cells and corroborated the observed co-stimulatory effect by transcriptional profiling analyses.

Comparison of H7N9 and H9N2 influenza infections in mouse model unravels the importance of early innate immune response in host protection.

The outcome of infection with influenza A virus is determined by a complex virus-host interaction. A new H7N9 virus of avian origin crossed the species barrier to infect humans, causing high mortality and emerged as a potential pandemic threat. The mechanisms underlying the virulence and pathogenicity of H7N9 virus remains elusive.

Prompt Antiviral Action of Pulmonary CD8+ T Cells Is Mediated by Rapid IFN-γ Induction and Its Downstream ISGs in the Lung.

Growing lines of evidence supported the importance of CD8+ lung tissue resident memory T (T) cells in protection against respiratory viruses, exemplified by influenza A virus. However, the underlying mechanism remains largely undetermined. Here, we used mouse infection models to dissect cross-protective activity of lung CD8+ T cells.

Temporal compressive imaging reconstruction based on a 3D-CNN network.

In temporal compressive imaging (TCI), high-speed object frames are reconstructed from measurements collected by a low-speed detector array to improve the system imaging speed. Compared with iterative algorithms, deep learning approaches utilize a trained network to reconstruct high-quality images in a short time. In this work, we study a 3D convolutional neural network for TCI reconstruction to make full use of the temporal and spatial correlation among consecutive object frames.

Hsa-miR-31 Governs T-Cell Homeostasis in HIV Protection IFN-γ-Stat1-T-Bet Axis.

It remains poorly defined whether any human miRNAs play protective roles during HIV infection. Here, focusing on a unique cohort of HIV-infected former blood donors, we identified miR-31 (hsa-miR-31) by comparative miRNA profiling as the only miRNA inversely correlating with disease progression. We further validated this association in two prospective cohort studies.

Conditioned CAR-T cells by hypoxia-inducible transcription amplification (HiTA) system significantly enhances systemic safety and retains antitumor efficacy.

Background: Hypoxia is a striking feature of most solid tumors and could be used to discriminate tumors from normoxic tissues. Therefore, the design of hypoxia-conditioned Chimeric Antigen Receptor (CAR) T cells is a promising strategy to reduce on-target off-tumor toxicity in adoptive cell therapy. However, existing hypoxia-conditioned CAR-T designs have been only partially successful in enhancing safety profile but accompanied with reduced cytotoxic efficacy.

The complete chloroplast genome of (Solanaceae: Petunioideae), an endangered ornamental species.

is an ornamental species on the brink of extinction in the wild. We report here the complete chloroplast genome of , which is 156,598 bp in size consisting of a large single-copy region (87,095 bp), a small single-copy region (18,643 bp), and a pair of inverted repeats (25,430 bp for each). The chloroplast (used 'cp' hereafter) genome contains 132 genes, including 8 rRNA genes, 37 tRNA genes, and 87 protein-coding genes.

High-resolution fast mid-wave infrared compressive imaging.

In the mid-wave infrared (MIR) band, large detector arrays are extremely costly and technically difficult to be manufactured. Thus, it is difficult to obtain high-resolution images for a conventional MIR camera. Spatial compressive imaging can improve resolution.

Broad dual-band temporal compressive imaging with optical calibration.

For applications such as remote sensing and bio-imaging, images from multiple bands can provide much richer information compared to a single band. However, most multispectral imaging systems have difficulty in acquiring images for high-speed moving objects. In this paper, we use a DMD-based temporal compressive imaging (TCI) system to obtain high-speed images of moving objects over a broad dual-band spectral range, in the visible and the near-infrared (NIR) bands simultaneously.

Gene Variants in Eight Chinese Patients With a Wide Range of Phenotypes.

The gene encodes the voltage-dependent P/Q-type calcium channel subunit alpha-1A, which is widely expressed throughout the CNS. The biological roles of the P/Q channel are diverse and the phenotypic spectrum caused by mutations is wide. The aim of this study is to demonstrate its phenotypic diversity and analyze the genotype-phenotype correlations in a cohort of Chinese patients.

Correlation Between Early Plasma Interleukin 37 Responses With Low Inflammatory Cytokine Levels and Benign Clinical Outcomes in Severe Acute Respiratory Syndrome Coronavirus 2 Infection.

Background: The immune protective mechanisms during severe acute respiratory syndrome coronavirus (SARS-CoV-2) infection remain to be deciphered for the development of an effective intervention approach.

Methods: We examined early responses of interleukin 37 (IL-37), a powerful anti-inflammatory cytokine, in 254 SARS-CoV-2-infected patients before any clinical intervention and determined its correlation with clinical prognosis.

Results: Our results demonstrated that SARS-CoV-2 infection causes elevation of plasma IL-37.

A Systemic Prime-Intrarectal Pull Strategy Raises Rectum-Resident CD8+ T Cells for Effective Protection in a Murine Model of LM-OVA Infection.

As the entry sites of many pathogens such as human immunodeficiency virus (HIV), mucosal sites are defended by rapidly reacting resident memory T cells (TRM). TRMs represent a special subpopulation of memory T cells that persist long term in non-lymphoid sites without entering the circulation and provide the "sensing and alarming" role in the first-line defense against infection. The rectum and vagina are the two primary mucosal portals for HIV entry.

CD160 Plays a Protective Role During Chronic Infection by Enhancing Both Functionalities and Proliferative Capacity of CD8+ T Cells.

The understanding of protective immunity during HIV infection remains elusive. Here we showed that CD160 defines a polyfunctional and proliferative CD8+ T cell subset with a protective role during chronic HIV-1 infection. CD160+ CD8+ T cells derived from HIV+ patients correlated with slow progressions both in a cross-sectional study and in a 60-month longitudinal cohort, displaying enhanced cytotoxicity and proliferative capacity in response to HIV Gag stimulation; triggering CD160 promoted their functionalities through MEK-ERK and PI3K-AKT pathways.

Percutaneous transforaminal endoscopic decompression for lumbar spinal stenosis with degenerative spondylolisthesis in the elderly.

Objectives: Percutaneous transforaminal endoscopic decompression (PTED) under local anesthesia is rarely performed for lumbar spinal stenosis (LSS) with degenerative lumbar spondylolisthesis (DLS) because of the limited field of vision, inherent instability, etc. The objective of this study was to describe the procedure of the PTED technique and to demonstrate the early clinical outcomes.

Patients And Methods: From January 2017 to January 2019, 40 consecutive patients aged 60 and older were diagnosed with LSS with DLS in our institution and underwent PTED.

Influenza Vaccine With Consensus Internal Antigens as Immunogens Provides Cross-Group Protection Against Influenza A Viruses.

Given that continuing antigenic shift and drift of influenza A viruses result in the escape from previous vaccine-induced immune protection, a universal influenza vaccine has been actively sought. However, there were very few vaccines capable of eliciting cross-group ant-influenza immunity. Here, we designed two novel composite immunogens containing highly conserved T-cell epitopes of six influenza A virus internal antigens, and expressed them in DNA, recombinant adenovirus-based (AdC68) and recombinant vaccinia vectors, respectively, to formulate three vaccine forms.

Molecular cloning, characterization of gene and its response to progesterone in .

Progesterone (P4) are aquatic contaminants that can impair fish reproduction even in low concentrations. The aim of this study was to investigate the effects of P4 on the sex differentiation, by quantitative determination of transcriptional changes of a candidate target gene (, has a function in the sex determination and gonadal differentiation of several vertebrate species) in . We first cloned and characterized the full-length cDNAs for the in (designated as ).

Molecular cloning and mRNA expression pattern of in .

, a member of the subfamily which of the Sox family, plays important roles in the development of the vertebrate gonad and nervous system. We have cloned a homologue fromthe brain of using homologous cloning and rapid amplification of cDNA ends. We named the cloned gene as .

TSC1 and DEPDC5 regulate HIV-1 latency through the mTOR signaling pathway.

The latent reservoir of HIV-1 presents a major barrier to viral eradication. The mechanism of the establishment and maintenance of the latent viral reservoir is not yet fully understood, which hinders the development of effective curative strategies. In this study, we identified two inhibitory genes, TSC1 and DEPDC5, that maintained HIV-1 latency by suppressing the mTORC1 pathway.

Targeting signal-transducer-and-activator-of-transcription 3 sensitizes human cutaneous melanoma cells to BRAF inhibitor.

Melanoma treatment with the BRAF V600E inhibitor vemurafenib provides therapeutic benefits but the common emergence of drug resistance remains a challenge. To define molecular mechanisms of vemurafenib resistance, we generated A375-R, WM35-R cell lines resistant to vemurafenib and show that the phosphorylated (p)-STAT3 was upregulated in these cells in vitro and in vivo. In particular, activation of the Signal-transducer-and-activator-of-transcription 3 (STAT3) pathway was associated with vemurafenib resistance.

Molecular cloning, characterization, and expression profiles of the Sox3 gene in Chinese loach Paramisgurnus dabryanus.

A number of studies have established that in vertebrates, Sox3 is involved in a wide range of developmental processes, including sex differentiation and neurogenesis. However, the exact functions of the Sox3 gene have not been documented so far in teleosts. Here, we cloned the full length cDNA of Sox3 from the teleost fish, Paramisgurnus dabryanus, which we designated PdSox3.

Molecular cloning and expression analysis of Sox3 during gonad and embryonic development in Misgurnus anguillicaudatus.

Sox3 is a single-exon gene located on the X chromosome in most vertebrates. It belongs to the SoxB1 subfamily, which is part of the larger Sox family. Previous studies have revealed that Sox3 is expressed in many fish species.