目的分析1例表现较为复杂的II型神经纤维瘤病(neurofibromatosis type II,NF2)患者的临床表现、听力康复方法和效果,明确一种特殊类型的NF2听神经瘤的听力重建方法及术后康复效果。方法整理该患者临床表现及既往史,术前检查,临床手术方...目的分析1例表现较为复杂的II型神经纤维瘤病(neurofibromatosis type II,NF2)患者的临床表现、听力康复方法和效果,明确一种特殊类型的NF2听神经瘤的听力重建方法及术后康复效果。方法整理该患者临床表现及既往史,术前检查,临床手术方法及术后随访听力康复效果。结果患者双侧听神经瘤,听力表现为双耳极重度感音神经性听力下降。外周血基因检测未见治病基因突变,考虑为嵌合体。患者右耳为内耳神经鞘膜瘤,且双眼视力极差,右耳行带瘤人工耳蜗植入术,术后通过为期2个月的以家庭为主的听力语言康复,听觉能力分级(CAP)由0分提高到5分。结论NF2临床表现复杂,临床预后差。带有微小的内耳神经鞘膜瘤的NF2患者可以通过带瘤植入人工耳蜗重建听力。展开更多
Angiotensin II (Ang II) is the main mediator of the Renin-Angiotensin-System acting on AT<sub>1</sub> and other AT receptors. It is regarded as a pleiotropic agent that induces many actions, including func...Angiotensin II (Ang II) is the main mediator of the Renin-Angiotensin-System acting on AT<sub>1</sub> and other AT receptors. It is regarded as a pleiotropic agent that induces many actions, including functioning as a growth factor, and as a contractile hormone, among others. The aim of this work was to examine the impact of Ang II on the expression and function of α<sub>1</sub>-adrenergic receptors (α<sub>1</sub>-ARs) in cultured rat aorta, and aorta-derived smooth muscle cells. Isolated Wistar rat aorta was incubated for 24 h in DMEM at 37˚C, then subjected to isometric tension and to the action of added norepinephrine, in concentration-response curves. Ang II was added (1 × 10<sup>−5</sup> M), and in some experiments, 5-Methylurapidil (α<sub>1A</sub>-AR antagonist), AH11110A (α<sub>1B</sub>-AR antagonist), or BMY-7378 (α<sub>1D</sub>-AR antagonist), were used to identify the α<sub>1</sub>-AR involved in the response. Desensitization of the contractile response to norepinephrine was observed due to incubation time, and by the Ang II action. α<sub>1D</sub>-AR was protected from desensitization by BMY-7378;while RS-100329 and prazosin partially mitigated desensitization. In another set of experiments, isolated aorta-derived smooth muscle cells were exposed to Ang II and α<sub>1</sub>-ARs proteins were evaluated. α<sub>1D</sub>-AR increased at 30 and 60 min post Ang II exposure, the α<sub>1A</sub>-AR diminished from 1 to 4 h, while α<sub>1B</sub>-AR remained unchanged over 24 h of Ang II exposure. Ang II induced an increase of α<sub>1D</sub>-AR at short times, and BMY-7378 protected α<sub>1D</sub>-AR from desensitization.展开更多
The catalysis of olefin polymerization through the chain-walking process is a subject of great interest. In this contribution, the successful synthesis of a Brookhart-type unsymmetrical α-diimine nickel catalyst Ni, ...The catalysis of olefin polymerization through the chain-walking process is a subject of great interest. In this contribution, the successful synthesis of a Brookhart-type unsymmetrical α-diimine nickel catalyst Ni, which contains both dibenzhydryl and phenyl groups, was determined by X-ray crystallography. The compound has a pseudo-tetrahedral geometry at the Ni center, showing pseudo-C2-symmetry. Upon activation with modified methylaluminoxane (MMAO), Ni1 exhibits high catalytic activity up to 1.02 × 107 g PE (mol Ni h)−1 toward ethylene polymerization, enabling the synthesis of high molecular weight branched polyethylene. The molecular weights and branching densities could be tuned over a very wide range. The polymerization results indicated the possibility of precise microstructure control, depending on the polymerization temperature. The branching densities were decreased with increasing the polymerization temperature.展开更多
In this study we used the deep eutectic solvents (ionic liquids) to investigate the reaction between copper (II) with ethylene diamine (en). Two of the existing methods for analyzing spectrophotometric measurements ha...In this study we used the deep eutectic solvents (ionic liquids) to investigate the reaction between copper (II) with ethylene diamine (en). Two of the existing methods for analyzing spectrophotometric measurements have been applied for establishing, the stoichiometry and whenever possible, the stability constants of the chelates formed. The method of continuous variations was necessary to determine first whether, the metal ion and the ligand ethylene diamine form one or more than one chelate, when more than one chelate formed, the results obtained depend on the wavelength and for meaningful conclusions the wavelengths were carefully selected. The empirical formulae of the chelates were further substantiated by the molar ratio method. The effect of time and temperature on the formation and stability of these chelates in solution is also studied. The stability constants, K1 and K2 for the copper (II) chelates were calculated, though reliable, and are comparable to literature values.展开更多
Carnitine Palmitoyl Transferase II (CPTII) is a very important enzyme that helps with the oxidation of long-chain fatty acid to produce energy. Deficiency in CPTII will lead to energy deficiency in the case of fasting...Carnitine Palmitoyl Transferase II (CPTII) is a very important enzyme that helps with the oxidation of long-chain fatty acid to produce energy. Deficiency in CPTII will lead to energy deficiency in the case of fasting and the accumulation of the long chain fatty in the body. There are three types of CPT II deficiency, the myopathic form, the severe infantile hepatocardiomuscular form and the lethal neonatal form. They are all inherited as an autosomal recessive. Diagnosis of the CPTII are 1) tandem mass spectrometry (MS/MS) in adult form and 2) CPTII polymorphism (F352C), which is linked to reducing the activity of CPTII in infantile form [1]. Glucose is the primary management and medium-chain fatty acid is an alternative due to the bypass of the CPTII enzyme in the pathway. For the prevention of CPTII deficiency are to avoid long chain fatty acid (C12-fatty acid), fasting, prolonged exercise, known triggers, and certain medications such as anti-epileptics and general anesthesia. During the rhabdomyolysis and myoglobinuria attack, it is very important to maintain hydration to avoid acute renal failure. If, however, renal failure occurs, dialysis is recommended. We present a case of a 27-year-old African American woman with the significant past medical history of CPT II deficiency leading to recurrent rhabdomyolysis and myoglobinuria. Together with all the research studies from diagnosis to treatment of CPTII deficiency will help in clinical management of patients. And this case report will add to the existing case reports of patients who have CPTII deficiency in terms of how we diagnose, how we treat, and how we prevent symptoms from re-occurring.展开更多
背景与目的目前,云南个旧锡矿有大量矿工从事开采工作,这种职业环境与接触粉尘颗粒、重金属、多环芳烃和放射性氡有关,大大增加了患肺癌的风险。本研究旨在探讨在云锡矿粉诱导大鼠肺泡Ⅱ型上皮细胞(immortalized rat alveolar cells ty...背景与目的目前,云南个旧锡矿有大量矿工从事开采工作,这种职业环境与接触粉尘颗粒、重金属、多环芳烃和放射性氡有关,大大增加了患肺癌的风险。本研究旨在探讨在云锡矿粉诱导大鼠肺泡Ⅱ型上皮细胞(immortalized rat alveolar cells type Ⅱ,RLE-6TN)恶性转化过程中,瘦素(leptin)及其介导的细胞外调节蛋白激酶(extracellular regulated protein kinase,ERK)信号通路所起的作用。方法采用200μg/mL的云锡矿粉隔代毒染RLE-6TN至第9代,建立毒染细胞模型,命名为R_(200)细胞,正常培养组命名为R细胞,通过Western blot法检测两种细胞leptin受体的表达情况。通过MTT法筛选出leptin及丝裂原活化蛋白激酶激酶(mitogen-activated protein kinase kinase,MEK)抑制剂(U0126)对R_(200)细胞的最佳作用浓度。自第20代起,将R组、R_(200)组细胞分别与leptin及MEK抑制剂U0126共培养,对各组细胞的形态改变进行观察,并利用苏木素-伊红(hematoxylin-eosin,HE)染色技术鉴别第40代细胞的形态学差异,通过刀豆凝集素A(concanavalin A,ConA)及锚着独立性生长实验法检测细胞恶性转化情况。通过Western blot法检测leptin作用后上皮细胞ERK信号通路的变化。结果R组和R_(200)组细胞均表达leptin受体(OB-R)。与R_(200)组比较,当leptin浓度达100 ng/mL时,其促增殖效应最为显著,30μmol/L U0126可抑制毒染细胞R_(200)增殖,与对照组相比具有统计学差异(P<0.05)。自第25代起,leptin诱导的R_(200)组(R_(200)L组)细胞形态发生变化,至第30代出现恶性转化,至第40代时恶性转化特征明显;而R_(200)组细胞及U0126诱导的R_(200)组(R_(200)LU组)细胞则在第40代时才出现恶性转化特征。R_(200)L组细胞凝集速度较R_(200)LU组快,其余各组细胞P30出现凝集,且随ConA浓度增加,细胞凝集速度加快。R_(200)L组细胞自P40可见克隆形成,克隆形成率为2.25‰±0.5‰,R_(200)LU组及R_(200)组未见克隆集落。R_(200)L组细胞pERK表达增强;加入U0126阻断后,R_(200)L组细胞pERK磷酸化水平降低。结论Leptin可以促进云锡矿粉毒染肺上皮细胞的恶性转化,ERK信号通路可能是其促进云锡矿粉引发的肺泡Ⅱ型上皮细胞转化的重要途径。展开更多
文摘目的分析1例表现较为复杂的II型神经纤维瘤病(neurofibromatosis type II,NF2)患者的临床表现、听力康复方法和效果,明确一种特殊类型的NF2听神经瘤的听力重建方法及术后康复效果。方法整理该患者临床表现及既往史,术前检查,临床手术方法及术后随访听力康复效果。结果患者双侧听神经瘤,听力表现为双耳极重度感音神经性听力下降。外周血基因检测未见治病基因突变,考虑为嵌合体。患者右耳为内耳神经鞘膜瘤,且双眼视力极差,右耳行带瘤人工耳蜗植入术,术后通过为期2个月的以家庭为主的听力语言康复,听觉能力分级(CAP)由0分提高到5分。结论NF2临床表现复杂,临床预后差。带有微小的内耳神经鞘膜瘤的NF2患者可以通过带瘤植入人工耳蜗重建听力。
文摘Angiotensin II (Ang II) is the main mediator of the Renin-Angiotensin-System acting on AT<sub>1</sub> and other AT receptors. It is regarded as a pleiotropic agent that induces many actions, including functioning as a growth factor, and as a contractile hormone, among others. The aim of this work was to examine the impact of Ang II on the expression and function of α<sub>1</sub>-adrenergic receptors (α<sub>1</sub>-ARs) in cultured rat aorta, and aorta-derived smooth muscle cells. Isolated Wistar rat aorta was incubated for 24 h in DMEM at 37˚C, then subjected to isometric tension and to the action of added norepinephrine, in concentration-response curves. Ang II was added (1 × 10<sup>−5</sup> M), and in some experiments, 5-Methylurapidil (α<sub>1A</sub>-AR antagonist), AH11110A (α<sub>1B</sub>-AR antagonist), or BMY-7378 (α<sub>1D</sub>-AR antagonist), were used to identify the α<sub>1</sub>-AR involved in the response. Desensitization of the contractile response to norepinephrine was observed due to incubation time, and by the Ang II action. α<sub>1D</sub>-AR was protected from desensitization by BMY-7378;while RS-100329 and prazosin partially mitigated desensitization. In another set of experiments, isolated aorta-derived smooth muscle cells were exposed to Ang II and α<sub>1</sub>-ARs proteins were evaluated. α<sub>1D</sub>-AR increased at 30 and 60 min post Ang II exposure, the α<sub>1A</sub>-AR diminished from 1 to 4 h, while α<sub>1B</sub>-AR remained unchanged over 24 h of Ang II exposure. Ang II induced an increase of α<sub>1D</sub>-AR at short times, and BMY-7378 protected α<sub>1D</sub>-AR from desensitization.
文摘The catalysis of olefin polymerization through the chain-walking process is a subject of great interest. In this contribution, the successful synthesis of a Brookhart-type unsymmetrical α-diimine nickel catalyst Ni, which contains both dibenzhydryl and phenyl groups, was determined by X-ray crystallography. The compound has a pseudo-tetrahedral geometry at the Ni center, showing pseudo-C2-symmetry. Upon activation with modified methylaluminoxane (MMAO), Ni1 exhibits high catalytic activity up to 1.02 × 107 g PE (mol Ni h)−1 toward ethylene polymerization, enabling the synthesis of high molecular weight branched polyethylene. The molecular weights and branching densities could be tuned over a very wide range. The polymerization results indicated the possibility of precise microstructure control, depending on the polymerization temperature. The branching densities were decreased with increasing the polymerization temperature.
文摘In this study we used the deep eutectic solvents (ionic liquids) to investigate the reaction between copper (II) with ethylene diamine (en). Two of the existing methods for analyzing spectrophotometric measurements have been applied for establishing, the stoichiometry and whenever possible, the stability constants of the chelates formed. The method of continuous variations was necessary to determine first whether, the metal ion and the ligand ethylene diamine form one or more than one chelate, when more than one chelate formed, the results obtained depend on the wavelength and for meaningful conclusions the wavelengths were carefully selected. The empirical formulae of the chelates were further substantiated by the molar ratio method. The effect of time and temperature on the formation and stability of these chelates in solution is also studied. The stability constants, K1 and K2 for the copper (II) chelates were calculated, though reliable, and are comparable to literature values.
文摘Carnitine Palmitoyl Transferase II (CPTII) is a very important enzyme that helps with the oxidation of long-chain fatty acid to produce energy. Deficiency in CPTII will lead to energy deficiency in the case of fasting and the accumulation of the long chain fatty in the body. There are three types of CPT II deficiency, the myopathic form, the severe infantile hepatocardiomuscular form and the lethal neonatal form. They are all inherited as an autosomal recessive. Diagnosis of the CPTII are 1) tandem mass spectrometry (MS/MS) in adult form and 2) CPTII polymorphism (F352C), which is linked to reducing the activity of CPTII in infantile form [1]. Glucose is the primary management and medium-chain fatty acid is an alternative due to the bypass of the CPTII enzyme in the pathway. For the prevention of CPTII deficiency are to avoid long chain fatty acid (C12-fatty acid), fasting, prolonged exercise, known triggers, and certain medications such as anti-epileptics and general anesthesia. During the rhabdomyolysis and myoglobinuria attack, it is very important to maintain hydration to avoid acute renal failure. If, however, renal failure occurs, dialysis is recommended. We present a case of a 27-year-old African American woman with the significant past medical history of CPT II deficiency leading to recurrent rhabdomyolysis and myoglobinuria. Together with all the research studies from diagnosis to treatment of CPTII deficiency will help in clinical management of patients. And this case report will add to the existing case reports of patients who have CPTII deficiency in terms of how we diagnose, how we treat, and how we prevent symptoms from re-occurring.
文摘背景与目的目前,云南个旧锡矿有大量矿工从事开采工作,这种职业环境与接触粉尘颗粒、重金属、多环芳烃和放射性氡有关,大大增加了患肺癌的风险。本研究旨在探讨在云锡矿粉诱导大鼠肺泡Ⅱ型上皮细胞(immortalized rat alveolar cells type Ⅱ,RLE-6TN)恶性转化过程中,瘦素(leptin)及其介导的细胞外调节蛋白激酶(extracellular regulated protein kinase,ERK)信号通路所起的作用。方法采用200μg/mL的云锡矿粉隔代毒染RLE-6TN至第9代,建立毒染细胞模型,命名为R_(200)细胞,正常培养组命名为R细胞,通过Western blot法检测两种细胞leptin受体的表达情况。通过MTT法筛选出leptin及丝裂原活化蛋白激酶激酶(mitogen-activated protein kinase kinase,MEK)抑制剂(U0126)对R_(200)细胞的最佳作用浓度。自第20代起,将R组、R_(200)组细胞分别与leptin及MEK抑制剂U0126共培养,对各组细胞的形态改变进行观察,并利用苏木素-伊红(hematoxylin-eosin,HE)染色技术鉴别第40代细胞的形态学差异,通过刀豆凝集素A(concanavalin A,ConA)及锚着独立性生长实验法检测细胞恶性转化情况。通过Western blot法检测leptin作用后上皮细胞ERK信号通路的变化。结果R组和R_(200)组细胞均表达leptin受体(OB-R)。与R_(200)组比较,当leptin浓度达100 ng/mL时,其促增殖效应最为显著,30μmol/L U0126可抑制毒染细胞R_(200)增殖,与对照组相比具有统计学差异(P<0.05)。自第25代起,leptin诱导的R_(200)组(R_(200)L组)细胞形态发生变化,至第30代出现恶性转化,至第40代时恶性转化特征明显;而R_(200)组细胞及U0126诱导的R_(200)组(R_(200)LU组)细胞则在第40代时才出现恶性转化特征。R_(200)L组细胞凝集速度较R_(200)LU组快,其余各组细胞P30出现凝集,且随ConA浓度增加,细胞凝集速度加快。R_(200)L组细胞自P40可见克隆形成,克隆形成率为2.25‰±0.5‰,R_(200)LU组及R_(200)组未见克隆集落。R_(200)L组细胞pERK表达增强;加入U0126阻断后,R_(200)L组细胞pERK磷酸化水平降低。结论Leptin可以促进云锡矿粉毒染肺上皮细胞的恶性转化,ERK信号通路可能是其促进云锡矿粉引发的肺泡Ⅱ型上皮细胞转化的重要途径。
