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Registros recuperados: 13
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A case of severe glutathione synthetase deficiency with novel GSS mutations BJMBR
Xia,H.; Ye,J.; Wang,L.; Zhu,J.; He,Z..
Glutathione synthetase deficiency (GSSD) is a rare inborn error of glutathione metabolism with autosomal recessive inheritance. The severe form of the disease is characterized by acute metabolic acidosis, usually present in the neonatal period with hemolytic anemia and progressive encephalopathy. A case of a male newborn infant who had severe metabolic acidosis with high anion gap, hemolytic anemia, and hyperbilirubinemia is reported. A high level of 5-oxoproline was detected in his urine and a diagnosis of generalized GSSD was made. DNA sequence analysis revealed the infant to be compound heterozygous with two mutations, c.738dupG in exon 8 of GSS gene resulting in p.S247fs and a repetitive sequence in exon 3 of GSS gene. Treatment after diagnosis of GSSD...
Tipo: Info:eu-repo/semantics/report Palavras-chave: Glutathione synthetase; 5-oxoprolinuria; Newborn; Metabolism; Mutation.
Ano: 2018 URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X2018000300501
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Analyzing gene expression profiles in dilated cardiomyopathy via bioinformatics methods BJMBR
Wang,Liming; Zhu,L.; Luan,R.; Wang,L.; Fu,J.; Wang,X.; Sui,L..
Dilated cardiomyopathy (DCM) is characterized by ventricular dilatation, and it is a common cause of heart failure and cardiac transplantation. This study aimed to explore potential DCM-related genes and their underlying regulatory mechanism using methods of bioinformatics. The gene expression profiles of GSE3586 were downloaded from Gene Expression Omnibus database, including 15 normal samples and 13 DCM samples. The differentially expressed genes (DEGs) were identified between normal and DCM samples using Limma package in R language. Pathway enrichment analysis of DEGs was then performed. Meanwhile, the potential transcription factors (TFs) and microRNAs (miRNAs) of these DEGs were predicted based on their binding sequences. In addition, DEGs were mapped...
Tipo: Info:eu-repo/semantics/article Palavras-chave: Dilated cardiomyopathy; Differentially expressed genes; Pathway enrichment analysis; Transcription factors; MicroRNAs; Small molecules.
Ano: 2016 URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X2016001000706
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Astragalus polysaccharides improve cardiomyopathy in STZ-induced diabetic mice and heterozygous (SOD2+/-) knockout mice BJMBR
Ju,J.; Chen,W.; Lai,Y.; Wang,L.; Wang,H.; Chen,W.J.; Zhao,X.; Ye,H.; LI,Y.; Zhang,Y..
Oxidative stress plays an important role in the development of diabetic cardiomyopathy. In the present study, we determined whether the effect of astragalus polysaccharides (APS) on diabetic cardiomyopathy was associated with its impact on oxidative stress. Streptozotocin (STZ)-induced diabetic mice and heterozygous superoxide dismutase (SOD2+/-) knockout mice were administered APS. The hemodynamics, cardiac ultrastructure, and the apoptosis, necrosis and proliferation of cardiomyocytes were assessed to evaluate the effect of APS on diabetic and oxidative cardiomyopathy. Furthermore, H2O2 formation, oxidative stress/damage, and SOD activity in cardiomyocytes were evaluated to determine the effects of APS on cardiac oxidative stress. APS therapy improved...
Tipo: Info:eu-repo/semantics/article Palavras-chave: Diabetes; Cardiomyopathy; Oxidative stress; Superoxide dismutase.
Ano: 2017 URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X2017000800601
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Changes in circulating endothelial progenitor cells predict responses of multiple myeloma patients to treatment with bortezomib and dexamethasone BJMBR
Wang,L.; Du,F.; Zhang,H.M.; Zhang,W.J.; Wang,H.X..
Four cycles of chemotherapy are required to assess responses of multiple myeloma (MM) patients. We investigated whether circulating endothelial progenitor cells (cEPCs) could be a biomarker for predicting patient response in the first cycle of chemotherapy with bortezomib and dexamethasone, so patients might avoid ineffective and costly treatments and reduce exposure to unwanted side effects. We measured cEPCs and stromal cell-derived factor-1α (SDF-1α) in 46 MM patients in the first cycle of treatment with bortezomib and dexamethasone, and investigated clinical relevance based on patient response after four 21-day cycles. The mononuclear cell fraction was analyzed for cEPC by FACS analysis, and SDF-1α was analyzed by ELISA. The study population was...
Tipo: Info:eu-repo/semantics/article Palavras-chave: Chemotherapy; Circulating endothelial progenitor cells; Multiple myeloma.
Ano: 2015 URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X2015000800736
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Establishment of patient-derived tumor xenograft (PDTX) models using samples from CT-guided percutaneous biopsy BJMBR
Zhuang,Y.-P.; Zhu,Y.-P.; Wang,H.-Y.; Sun,L.; Zhang,J.; Hao,Y.-P.; Wang,L..
This study aimed to investigate the feasibility of the establishment of a human cancer xenograft model using samples from computed tomography (CT)-guided percutaneous biopsy. Fresh tumor tissues obtained from 10 cancer patients by CT-guided percutaneous biopsy were subcutaneously inoculated into NOD-Prkdcem26Il2rgem26Nju (NCG) mice to establish human patient-derived tumor xenograft (PDTX) models. The formation of first and second generation xenografts was observed, and tumor volume was recorded over time. Tumor tissue consistency between the PDTX model and primary tumors in patients was compared using H&E staining and immunohistochemistry. Pharmacodynamic tests of clinically used chemotherapeutic drugs were conducted on second generation xenografts,...
Tipo: Info:eu-repo/semantics/article Palavras-chave: Cancer; Patient-derived tumor xenografts (PDTX); Computed tomography; X-ray; Percutaneous biopsy; Mouse models.
Ano: 2017 URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X2017000600705
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Evaluation of a father and son with atypical chronic myeloid leukemia with SETBP1 mutations and a review of the literature BJMBR
Wang,L.; Du,F.; Zhang,H.-M.; Wang,H.-X..
We report the case of a father and son diagnosed with atypical chronic myeloid leukemia (aCML). Both patients harbored SETBP1 mutations, which are present in 24.3% of aCML patients. Moreover, both shared the variant encoding p.Pro737His, but the aCML severity was greater in the son because of the presence of two other missense mutations causing p.Asp868Asn and p.Ser885Arg alterations. SETBP1 mutations may be associated with an adverse prognosis, so their detection would help in the diagnosis of aCML and the determination of a patient's prognosis.
Tipo: Info:eu-repo/semantics/article Palavras-chave: Atypical chronic myeloid leukemia; SETBP1 mutation; Chronic neutrophilic leukemia; CSF3R mutation.
Ano: 2015 URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X2015000700583
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Glucagon-like peptide 1 improves insulin resistance in vitro through anti-inflammation of macrophages BJMBR
Guo,C.; Huang,T.; Chen,A.; Chen,X.; Wang,L.; Shen,F.; Gu,X..
Glucagon-like peptide 1 (GLP-1), a kind of gut hormone, is used in the treatment of type 2 diabetes (T2D). Emerging evidence indicates that GLP-1 has anti-inflammatory activity. Chronic inflammation in the adipose tissue of obese individuals is a cause of insulin resistance and T2D. We hypothesized that GLP-1 analogue therapy in patients with T2D could suppress the inflammatory response of macrophages, and therefore inhibit insulin resistance. Our results showed that GLP-1 agonist (exendin-4) not only attenuated macrophage infiltration, but also inhibited the macrophage secretion of inflammatory cytokines including TNF-β, IL-6, and IL-1β. Furthermore, we observed that lipopolysaccharide (LPS)-induced macrophage conditioned media could impair...
Tipo: Info:eu-repo/semantics/article Palavras-chave: Diabetes; Glucagon-like peptide; Macrophage infiltration; Adipose inflammation; Insulin resistance.
Ano: 2016 URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X2016001200602
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Mutations in the HFE gene and sporadic amyotrophic lateral sclerosis risk: a meta-analysis of observational studies BJMBR
Li,M.; Wang,L.; Wang,W.; Qi,X.L.; Tang,Z.Y..
Iron homeostasis dysregulation has been regarded as an important mechanism in neurodegenerative diseases. The H63D and C282Y polymorphisms in theHFE gene may be involved in the development of sporadic amyotrophic lateral sclerosis (ALS) through the disruption of iron homeostasis. However, studies investigating the relationship between ALS and these two polymorphisms have yielded contradictory outcomes. We performed a meta-analysis to assess the roles of the H63D and C282Y polymorphisms of HFEin ALS susceptibility. PubMed, MEDLINE, EMBASE, and Cochrane Library databases were systematically searched to identify relevant studies. Strict selection criteria and exclusion criteria were applied. Odds ratios (ORs) with 95% confidence intervals (CIs) were used to...
Tipo: Info:eu-repo/semantics/article Palavras-chave: Amyotrophic lateral sclerosis; HFE; C282Y; H63D; Polymorphism; Meta-analysis.
Ano: 2014 URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X2014000300215
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Poly(3-hydroxybutyrate-co-3-hydroxyhexanoate)-based scaffolds for tissue engineering BJMBR
Chang,H.M.; Wang,Z.H.; Luo,H.N.; Xu,M.; Ren,X.Y.; Zheng,G.X.; Wu,B.J.; Zhang,X.H.; Lu,X.Y.; Chen,F.; Jing,X.H.; Wang,L..
Development and selection of an ideal scaffold is of importance for tissue engineering. Poly(3-hydroxybutyrate-co-3-hydroxyhexanoate) (PHBHHx) is a biocompatible bioresorbable copolymer that belongs to the polyhydroxyalkanoate family. Because of its good biocompatibility, PHBHHx has been widely used as a cell scaffold for tissue engineering. This review focuses on the utilization of PHBHHx-based scaffolds in tissue engineering. Advances in the preparation, modification, and application of PHBHHx scaffolds are discussed.
Tipo: Info:eu-repo/semantics/article Palavras-chave: PHBHHx; Tissue engineering; Biomaterial.
Ano: 2014 URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X2014000700533
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Quercetin alleviates pulmonary angiogenesis in a rat model of hepatopulmonary syndrome BJMBR
Li,X.; Chen,Y.; Wang,L.; Shang,G.; Zhang,C.; Zhao,Z.; Zhang,H.; Liu,A..
Quercetin shows protective effects against hepatopulmonary syndrome (HPS), as demonstrated in a rat model. However, whether these effects involve pulmonary vascular angiogenesis in HPS remains unclear. Therefore, this study aimed to assess the effect of quercetin on pulmonary vascular angiogenesis and explore the underlying mechanisms. Male Sprague-Dawley rats weighing 200-250 g underwent sham operation or common bile duct ligation (CBDL). Two weeks after surgery, HIF-1α and NFκB levels were assessed in rat lung tissue by immunohistochemistry and western blot. Then, CBDL and sham-operated rats were further divided into 2 subgroups each to receive intraperitoneal administration of quercetin (50 mg/kg daily) or 0.2% Tween for two weeks: Sham (Sham+Tween;...
Tipo: Info:eu-repo/semantics/article Palavras-chave: Angiogenesis; Hepatopulmonary syndrome; HIF-1α; Quercetin.
Ano: 2016 URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X2016000700606
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The anesthetic agent sevoflurane attenuates pulmonary acute lung injury by modulating apoptotic pathways BJMBR
Wang,L.; Ye,Y.; Su,H.B.; Yang,J.P..
The objective of this study was to evaluate lung protection by the volatile anesthetic sevoflurane (SEVO), which inhibits apoptosis. Male Sprague-Dawley rats (250–280 g; n=18) were randomly divided into three groups. The LPS group received 5 mg/kg endotoxin (lipopolysaccharide), which induced acute lung injury (ALI). The control (CTRL) group received normal saline and the SEVO group received sevoflurane (2.5%) for 30 min after ALI was induced by 5 mg/kg LPS. Samples were collected for analysis 12 h after LPS. Lung injury was assessed by pathological observations and tissue wet to dry weight (W/D) ratios. Apoptotic index (AI) was determined by terminal deoxynucleotidyl transferase dUTP nick-end labeling (TUNEL) assay and electron microscopy. Caspase-3 and...
Tipo: Info:eu-repo/semantics/article Palavras-chave: Sevofluran; Apoptosis; Pulmonary acute lung injury; Caspase-3; Bcl-2.
Ano: 2017 URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X2017000300702
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Treatment of a subdural empyema complicated by intracerebral abscess due to Brucella infection BJMBR
Zhang,J.; Chen,Z.; Xie,L.; Zhao,C.; Zhao,H.; Fu,C.; Chen,G.; Hao,Z.; Wang,L.; Li,W..
A 55-year-old male presented with fever, stupor, aphasia, and left hemiparesis. A history of head trauma 3 months before was also reported. Cranial magnetic resonance imaging revealed slight contrast enhancement of lesions under the right frontal skull plate and right frontal lobe. Because of deterioration in nutritional status and intracranial hypertension, the patient was prepared for burr hole surgery. A subdural empyema (SDE) recurred after simple drainage. After detection of Brucella species in SDE, craniotomy combined with antibiotic treatment was undertaken. The patient received antibiotic therapy for 6 months (two doses of 2 g ceftriaxone, two doses of 100 mg doxycycline, and 700 mg rifapentine for 6 months) that resulted in complete cure of the...
Tipo: Info:eu-repo/semantics/report Palavras-chave: Subdural empyema; Neurobrucellosis; Craniotomy; Infection; Treatment.
Ano: 2017 URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X2017000500501
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Two novel Mg(II)-based and Zn(II)-based complexes: inhibiting growth of human liver cancer cells BJMBR
Liu,N.; Ding,D.; Wang,L.; Zhao,H.; Zhu,L.; Geng,X..
Two new Mg(II)-based and Zn(II)-based coordination polymers, {[Mg3(BTB)(DMA)4](DMA)2}n (1, H3BTB=1,3,5-benzenetrisbenzoic acid, DMA=N,N-dimethylacetamide) and {(H2NMe2)2[Zn3(BTB)2(OH)(Im)](DMF)9(MeOH)7}n (2, Im=imidazole, DMF=N,N-dimethylformamide), have been successfully synthesized and structurally characterized under solvothermal conditions. 1 contains a linear [Mg3(COO)6] cluster that connected by the fully deprotonated BTB3- ligands to give a kgd-type 2D bilayer structure; 2 represents a microporous 3D pillar-layered system based on the binuclear Zn units and pillared Im ligands, which shows a (3,5)-connected hms topological net. In addition, in vitro anticancer activities of compounds 1 and 2 on 4 human liver cancer cells (HB611, HHCC, BEL-7405 and...
Tipo: Info:eu-repo/semantics/article Palavras-chave: Coordination polymer; Pillar-layered; Liver cancer.
Ano: 2018 URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X2018000200614
Registros recuperados: 13
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