Research hotspots and trends for Duchenne muscular dystrophy: a machine learning bibliometric analysis from 2004 to 2023.

Aims: The aim of this study was to conduct a bibliometric analysis of the relevant literature on Duchenne muscular dystrophy (DMD) to ascertain its current status, identify key areas of research and demonstrate the evolution of the field.

Methods: The analysis sourced documents from the Science Citation Index Expanded in the Web of Science core collection, utilizing CiteSpace software and an online bibliometric platform to analyze collaborative networks among authors, institutions and countries, and to map out the research landscape through journal and reference evaluations. Keyword analyses, including clustering and emergent term identification, were conducted, alongside the development of knowledge maps.

TBK1 is involved in M-CSF-induced macrophage polarization through mediating the IRF5/IRF4 axis.

TANK binding kinase 1 (TBK1) is an important kinase that is involved in innate immunity and tumor development. Macrophage colony-stimulating factor (M-CSF) regulates the differentiation and function of macrophages towards the immunosuppressive M2 phenotype in the glioblastoma multiforme microenvironment. The role of TBK1 in macrophages, especially in regulating macrophage polarization in response to M-CSF stimulation, remains unclear.

Inhibiting NF-κB inducing kinase improved the motor performance of ALS animal model.

Background: Amyotrophic lateral sclerosis (ALS) is a typical neurodegenerative disorder typically characterized by inflammation activation. However, the relationship between non-canonical NF-κB (ncNF-κB) pathway activation and ALS progression is not clear.

Methods: We tested the ncNF-κB pathway in the ALS animal model including hSOD1-G93A transgenic mice and TBK1 deletion mice.

Upregulation of HLA-II related to LAG-3CD4 T cell infiltration is associated with patient outcome in human glioblastoma.

Glioblastoma (GBM) is the most common malignant diffuse glioma of the brain. Although immunotherapy with immune checkpoint inhibitors (ICIs), such as programmed cell death protein (PD)-1/PD ligand-1 inhibitors, has revolutionized the treatment of several cancers, the clinical benefit in GBM patients has been limited. Lymphocyte-activation gene 3 (LAG-3) binding to human leukocyte antigen-II (HLA-II) plays an essential role in triggering CD4 T cell exhaustion and could interfere with the efficiency of anti-PD-1 treatment; however, the value of LAG-3-HLA-II interactions in ICI immunotherapy for GBM patients has not yet been analyzed.

Progress on the hippocampal circuits and functions based on sharp wave ripples.

Sharp wave ripples (SWRs) are high-frequency synchronization events generated by hippocampal neuronal circuits during various forms of learning and reactivated during memory consolidation and recall. There is mounting evidence that SWRs are essential for storing spatial and social memories in rodents and short-term episodic memories in humans. Sharp wave ripples originate mainly from the hippocampal CA3 and subiculum, and can be transmitted to modulate neuronal activity in cortical and subcortical regions for long-term memory consolidation and behavioral guidance.

IRF5 knockdown reverses TDP-related phenotypes partially by increasing TBK1 expression.

Interferon-regulatory factor 5 (IRF5) participates in the regulation of apoptosis, affects the phenotype of inflammatory macrophages and plays an essential role in the inflammatory response. However, the role of IRF5 in the progression of amyotrophic lateral sclerosis (ALS) remains largely unknown. Here, we show that IRF5 mainly accumulated in the nucleus in cells expressing the truncated 25 k C-terminal fragments of TDP-43 (TDP-25, named TDP-25 cells hereafter).

Normal and Abnormal Sharp Wave Ripples in the Hippocampal-Entorhinal Cortex System: Implications for Memory Consolidation, Alzheimer's Disease, and Temporal Lobe Epilepsy.

The appearance of hippocampal sharp wave ripples (SWRs) is an electrophysiological biomarker for episodic memory encoding and behavioral planning. Disturbed SWRs are considered a sign of neural network dysfunction that may provide insights into the structural connectivity changes associated with cognitive impairment in early-stage Alzheimer's disease (AD) and temporal lobe epilepsy (TLE). SWRs originating from hippocampus have been extensively studied during spatial navigation in rodents, and more recent studies have investigated SWRs in the hippocampal-entorhinal cortex (HPC-EC) system during a variety of other memory-guided behaviors.

Deletion of FGF9 in GABAergic neurons causes epilepsy.

Fibroblast growth factor 9 (FGF9) has long been assumed to modulate multiple biological processes, yet very little is known about the impact of FGF9 on neurodevelopment. Herein, we found that loss of Fgf9 in olig1 progenitor cells induced epilepsy in mice, with pathological changes in the cortex. Then depleting Fgf9 in different neural populations revealed that epilepsy was associated with GABAergic neurons.

Myeloid TBK1 Deficiency Induces Motor Deficits and Axon Degeneration Through Inflammatory Cell Infiltration.

Background: TANK-binding kinase1 (TBK1) haploinsufficiency has been shown to cause both amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD); however, the mechanism is unclear.

Methods: Myeloid Tbk1 knockout mice (Tbk1-LKO mice) were established and motor function and pathological analyses were also performed. The level of p-TBK1 was analyzed in the ALS animal model and in patient samples using flow cytometry.

Myeloid NEMO deficiency promotes tumor immunosuppression partly via MCP1-CCR2 axis.

Tumor-associated macrophages (TAM), which are found in the tumor microenvironment of solid tumors, not only mediate cancer immune evasion but also promote tumor growth. The transcription factor NF-κB, which is a crucial link between inflammation and tumors, can accelerate tumor occurrence and development. NEMO, the regulatory subunit of the IKK complex, plays a pivotal role in activating the NF-κB signaling pathway.

The deletion of mutant SOD1 via CRISPR/Cas9/sgRNA prolongs survival in an amyotrophic lateral sclerosis mouse model.

The superoxide dismutase 1 (SOD1) mutation is one of the most notable causes of amyotrophic lateral sclerosis (ALS), and modifying the mutant SOD1 gene is the best approach for the treatment of patients with ALS linked to the mutations in this gene. Clustered regularly interspaced short palindromic repeat (CRISPR)/CRISPR-associated (Cas9)/sgRNA delivered by the adeno-associated virus (AAV) system is a powerful tool for genome editing in the central nervous system (CNS). Here, we tested the capacity of the AAV-SaCas9-sgRNA system to modify mutant SOD1 in SOD1G93A transgenic mice and found that AAV9-SaCas9-sgRNA5 deleted the SOD1 gene, improved the lifespan of SOD1G93A mice by 54.

FGF9 knockout in GABAergic neurons induces apoptosis and inflammation via the Fas/caspase-3 pathway in the cerebellum of mice.

Fibroblast growth factor 9 (FGF9) is a member of the fibroblast growth factor family and is widely expressed in the central nervous system (CNS). However, it is not clear how the working mechanism of FGF9 is involved in cerebellar development. To address this question, we deleted the Fgf9 gene specifically in GABAergic neurons or glutamatergic neurons, and demonstrated that Fgf9 ablation in GABAergic neurons rather than the glutamatergic neurons caused severe ataxia.

Deletion of Tbk1 disrupts autophagy and reproduces behavioral and locomotor symptoms of FTD-ALS in mice.

Haploinsufficiency of the protein kinase Tbk1 has shown to cause both amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD); however, the pathogenic mechanisms are unclear. Here we show that conditional neuronal deletion of Tbk1 in leads to cognitive and locomotor deficits in mice. Tbk1-NKO mice exhibited numerous neuropathological changes, including neurofibrillary tangles, abnormal dendrites, reduced dendritic spine density, and cortical synapse loss.

[Raman spectra calculation and analysis of plasticizer dioctyl phthalate].

In recent years, with frequent domestic food safety incidents related to the plasticizing agent, the detection of plasticizers in food research becomes increasingly urgent. DEHP is one of the plasticizer. In the present paper, theoretical Raman spectrum and experimental Raman spectrum of DEHP were given.

Burdock fructooligosaccharide induces stomatal closure in Pisum sativum.

Burdock fructooligosaccharide (BFO) isolated from the root tissue of Arctium lappa is a reserve carbohydrate that can induce resistance against a number of plant diseases. Stomatal closure is a part of plant innate immune response to restrict bacterial invasion. In this study, the effects of BFO on stomata movement in Pisum sativum and the possible mechanisms were studied with abscisic acid (ABA) as a positive control.

Phospholipid micelle-based magneto-plasmonic nanoformulation for magnetic field-directed, imaging-guided photo-induced cancer therapy.

Unlabelled: We present a magnetoplasmonic nanoplatform combining gold nanorods (GNR) and iron-oxide nanoparticles within phospholipid-based polymeric nanomicelles (PGRFe). The gold nanorods exhibit plasmon resonance absorbance at near infrared wavelengths to enable photoacoustic imaging and photothermal therapy, while the Fe3O4 nanoparticles enable magnetophoretic control of the nanoformulation. The fabricated nanoformulation can be directed and concentrated by an external magnetic field, which provides enhancement of a photoacoustic signal.

Furry pet allergens, fungal DNA and microbial volatile organic compounds (MVOCs) in the commercial aircraft cabin environment.

There has been concern about the cabin environment in commercial aircraft. We measured cat, dog and horse allergens and fungal DNA in cabin dust and microbial volatile organic compounds (MVOCs) in cabin air. Samples were collected from two European airline companies, one with cabins having textile seats (TSC) and the other with cabins having leather seats (LSC), 9 airplanes from each company.

Burdock fructooligosaccharide induces fungal resistance in postharvest Kyoho grapes by activating the salicylic acid-dependent pathway and inhibiting browning.

Burdock fructooligosaccharide (BFO) is a natural elicitor from Arcitum lappa. The effects of BFO in controlling postharvest disease in grape, apple, banana, kiwi, citrus, strawberry, and pear were investigated. The disease index, decay percentage, and area under the disease progress curve indicated that BFO has general control effects on postharvest disease of fruits.

Transcriptome profile analysis of resistance induced by burdock fructooligosaccharide in tobacco.

Burdock fructooligosaccharide (BFO), isolated from the roots of Arcitum lappa, is a novel potential elicitor. Previous studies have shown that BFO induces various defense responses in plants. However, little is known about the mechanism of BFO induced plant responses.

[Optimization of melamine structure using density functional theory and vibrational spectra studies].

Melamine was used in foodstuff and feed industry as a feed additive occasionally. In the present work, melamine geometry structure was optimized by density functional theory (DFT) method. Raman and infrared spectra were calculated based on MP2/6-31G sets and DFT/DGTIVP sets, and then two theoretical Raman spectra were carefully compared with other experimental spectra.

[Raman scattering calculations studies of malachite green isothiocyanate].

Malachite green isothiocyanate (MGITC) is a Raman probe molecule that was applied to cells detection, tissue composition detection and cells stain imaging. In the present work, MGITC molecular structure was optimized by density functional theory(DFT) calculation. MGITC molecular Raman spectra and infrared spectra were calculated with Hartree-Fork theory and MP2 theory based on STO-3G level, then two theoretical Raman spectra were carefully compared with experimental spectra, and good agreements were obtained between the theoretical and experimental results.