OTULIN deficiency: focus on innate immune system impairment.

OTULIN deficiency is a complex disease characterized by a wide range of clinical manifestations, including skin rash, joint welling, lipodystrophy to pulmonary abscess, and sepsis shock. This disease is mechanistically linked to mutations in the gene, resulting in an immune disorder that compromises the body's ability to effectively combat pathogens and foreign stimuli. The gene is responsible for encoding a deubiquitinating enzyme crucial for hydrolyzing Met1-poly Ub chains, and its dysfunction leads to dysregulated immune responses.

Reconstructive strategies following surgical resection of malignant sublingual gland tumors: A single institution experience.

Objective: To investigate the characteristics and treatment modalities of malignant tumors originating from the sublingual gland, as well as evaluate the therapeutic outcomes following free flap reconstruction.

Methods: A retrospective statistical analysis was conducted on the clinical data of nine patients diagnosed with malignant neoplasms tumor of the sublingual gland.

Results: Nine case of malignant tumors originated from the sublingual glandular tissue, encompassing eight adenoid cystic carcinoma (ACC) and a single case of bipartite differentiated carcinoma-a hybrid of epithelial-myoepithelial carcinoma and adenoid cystic carcinoma.

Circ_0001666 upregulation promotes intestinal epithelial cell fibrosis in pediatric Crohn's disease via the SRSF1/BMP7 axis.

The epithelial-mesenchymal transition (EMT) is closely associated with Crohn's disease (CD) related intestinal fibrosis, a condition whose prevalence is increasing annually among children. Recently, the CD marker gene microarray screening revealed an upregulation of circ_0001666 in the colon tissues of CD patients, but its underlying mechanisms remain unclear. In this study, we explored the molecular mechanism of circ_0001666 in regulating EMT-mediated fibrosis in CD in vitro.

Use of the Endoleak-to-Aortic Density Ratio to Distinguish Direct Endoleaks from Indirect Endoleaks after Endovascular Aortic Aneurysm Repair.

Purpose: To define criteria to distinguish direct (type 1 or 3) from indirect endoleaks (type 2) in the arterial phase of contrast-enhanced computed tomography (CT) scans in patients with abdominal aortic aneurysms treated with endovascular aortic repair.

Materials And Methods: This retrospective study was conducted from January 2009 to October 2020 and included consecutive patients treated endovascularly for a direct endoleak or an indirect endoleak associated with an enlarging aneurysm. The following characteristics were evaluated using contrast-enhanced CT: location, size, contact with the endograft, density, morphologic criteria, collateral artery enhancement, and endoleak-to-aortic density ratio.

Cardioprotective effects of circ_0002612 in myocardial ischemia/reperfusion injury correlate with disruption of miR-30a-5p-dependent Ppargc1a inhibition.

Introduction: Novel mechanistic insights into the effects of circular RNAs (circRNAs) on the physiology and pathology of cardiovascular diseases are under increasingly active investigation. This study defined the cardioprotective role and mechanistic actions of circ_0002612 in myocardial ischemia/reperfusion injury (MI/RI).

Methods: MI/RI was induced in mice by ligation of the left anterior descending (LAD) artery followed by reperfusion, and the in vitro model was established in cultured cardiomyocytes under hypoxia/reoxygenation (H/R) conditions.

CircZNF609 Aggravated Myocardial Ischemia Reperfusion Injury via Mediation of miR-214-3p/PTGS2 Axis.

Background And Objectives: Circular RNAs were known to play vital role in myocardial ischemia reperfusion injury (MIRI), while the role of CircZNF609 in MIRI remains unclear. This study was aimed to investigate the function of CircZNF609 in MIRI.

Methods: Hypoxia/reoxygenation (H/R) model was established to mimic MIRI .

Jieduan-Niwan Formula Ameliorates Oxidative Stress and Apoptosis in Acute-on-Chronic Liver Failure by Suppressing HMGB1/TLR-4/NF-B Signaling Pathway: A Study In Vivo and In Vitro.

Jieduan-Niwan (JDNW) formula is a traditional Chinese medicine compound created by the famous Chinese medicine expert Professor Qian Ying, and has been used clinically for decades to treat acute-on-chronic liver failure (ACLF) and exhibits remarkable efficacy. However, the exact mechanism remains to be discovered. As an important hepatocyte damage-associated molecular patterns (DAMP) factor, high mobility group box 1 (HMGB1) is a potential therapeutic target as an accelerator of ACLF in the pathogenesis.

Quercetin Reduces Oxidative Stress and Apoptosis by Inhibiting HMGB1 and Its Translocation, Thereby Alleviating Liver Injury in ACLF Rats.

Background: Acute on chronic liver failure (ACLF) is a syndrome of acute liver failure that occurs on the basis of chronic liver disease, which is characterized by a rapid deterioration in a short period and high mortality. High mobility group box 1 (HMGB1) may be involved in the pathological process of ACLF; its specific role remains to be further elucidated. Our previous studies have shown that quercetin (Que) exerts anti-oxidant and anti-apoptotic effects by inhibiting HMGB1 in vitro.

Network Pharmacology-Based Analysis on the Potential Biological Mechanisms of Sinisan Against Non-Alcoholic Fatty Liver Disease.

Non-alcoholic fatty liver disease (NAFLD) has become the most prevalent liver disease in China. Sinisan (SNS) is a traditional Chinese medicine formula that has been widely used in treating chronic liver diseases, including NAFLD. However, its underlying biological mechanisms are still unclear.

Paclitaxel and mortality in patients with claudication and de novo femoropopliteal lesions: a historical cohort study.

Objective: To compare the mortality rates of patients with claudication and de novo femoropopliteal lesions treated with and without paclitaxel coated devices (PCD).

Background: A recent meta-analysis, mostly including patients with claudication and de novo femoropopliteal lesions but also with recurrent stenoses and critical limb ischemia, has shown a significant excess mortality in patients treated with PCD.

Methods: Comparison of two historical cohorts of patients presenting with claudication and de novo femoropopliteal lesions treated with and without PCD between 2008 and 2018.

XBP1- IGFBP3 Signaling Pathway Promotes NSCLC Invasion and Metastasis.

Lung cancer is the most frequently diagnosed cancer and the main cause of cancer death in the world. X-box binding protein 1 (XBP1), which is an important transcription factor involved in regulating the unfolded protein response (UPR) during endoplasmic reticulum (ER) stress, might act as a potent oncogenic protein in the processes of tumorigenesis, tumor proliferation and metastasis in various cancers. However, the clinical significance and pathological role of XBP1 in non-small cell lung cancer (NSCLC) remains unknown.

The roles of metastasis-related proteins in the development of giant cell tumor of bone, osteosarcoma and Ewing's sarcoma.

Background: Giant cell tumor of bone (GC), osteosarcoma (OS) and Ewing's sarcoma (ES) are three different types of bone cancer with common and specific pathology features.

Objective: The purpose of the study was to examine the relationship and differences of the three bone tumors using clinical samples.

Methods: Through screening the profiles of clinical samples from GC, OS and ES patients using a humanoncology array, we found 26, 25 and 15 tumorigenesis factors significantly increased in GS, OS and ES tissues compared to normal individuals.

The Effect of HRE-Regulated VEGF Expression and Transfection on Neural Stem Cells in Rats.

In this study, we analyzed neural stem cells transfected with the HRE-VEGF gene in groups experiencing different periods of hypoxia. The results of RT-PCR showed that the expression of vascular endothelial growth factor (VEGF) mRNA gradually increased with the prolonged period of hypoxia ( < 0.05).

[Hereditary hemorrhagic telangiectasia: a report of two cases].

This article reports two children with hereditary hemorrhagic telangiectasia (HHT). Patient 1 was a boy aged 12 years and was admitted due to intermittent cough and wheezing for more than 10 years. This boy and his mother and grandmother had a history of epistaxis.

Association Between the Use of Cannabis and Physical Violence in Youths: A Meta-Analytical Investigation.

Objective: The aim of this meta-analysis was to investigate the extent to which cannabis use among youths is associated with the risk of perpetrating physical violence.

Methods: Searches were conducted in PubMed, PsycINFO, Web of Science, and Google Scholar for articles published from the inception of each database to July 2019. All studies that examined both cannabis use and the perpetration of physical violence in a sample of youths and young adults <30 years old were included.

Anti-inflammation of Erianin in dextran sulphate sodium-induced ulcerative colitis mice model via collaborative regulation of TLR4 and STAT3.

Ulcerative colitis (UC) is a chronic, idiopathic and inflammatory disease of the rectal and colonic mucosa. Studies have shown that Toll-like receptors (TLR) 4 and Signal Transducer and Activator of Transcription 3 (STAT3)-mediated the decline in immune function and inflammatory infiltration are potential pathomechanism of UC occurrence and development. In this study, the anti-inflammation of Erianin, a natural bibenzyl compound with the antioxidant, antitumor, and anti-inflammatory activities, was investigated in a dextran sodium sulphate-induced UC mouse model.

Tumor microenvironment-driven non-cell-autonomous resistance to antineoplastic treatment.

Drug resistance is of great concern in cancer treatment because most effective drugs are limited by the development of resistance following some periods of therapeutic administration. The tumor microenvironment (TME), which includes various types of cells and extracellular components, mediates tumor progression and affects treatment efficacy. TME-mediated drug resistance is associated with tumor cells and their pericellular matrix.

mTOR: An attractive therapeutic target for osteosarcoma?

Osteosarcoma (OS) is a common primary malignant bone tumor with high morbidity and mortality in children and young adults. How to improve poor prognosis of OS due to resistance to chemotherapy remains a challenge. Recently, growing findings show activation of mammalian target of rapamycin (mTOR), is associated with OS cell growth, proliferation, metastasis.

Functional analysis of β-amyrin synthase gene in ginsenoside biosynthesis by RNA interference.

Down-regulation of β-amyrin synthase gene expression by RNA interference led to reduced levels of β-amyrin and oleanane-type ginsenoside as well as up-regulation of dammarane-type ginsenoside level. In the biosynthetic pathway of ginsenosides, β-amyrin synthase catalyzes the reaction from oxidosqualene to β-amyrin, the proposed aglycone of oleanane-type saponins. Here, RNAi was employed to evaluate the role of this gene in ginsenoside biosynthesis of Panax ginseng hairy roots.

[Effect of triptolide on allogenic tendon transplantation in repairing tendon defect in chicken].

Objective: Triptolide can suppress immunological rejection reaction. To investigate the effect of triptolide on allogenic tendon transplantation in repairing tendon defect in chicken.

Methods: The defect model of the third toes tendon was established in 64 healthy-cleaning male Leghorn chickens (4-month-old, weighing 1.