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| 1 | Hepatogenic differentiation of human mesenchymal stem cells from adipose tissue in comparison with bone marrow mesenchymal stem cells显示文摘AIM: To investigate and compare the hepatogenic transdifferentiation of adipose tissue-derived stem cells (ADSC) and bone marrow-derived mesenchymal stem cells (BMSC) in vitro. Transdifferentiation of BMSC into hepatic cells in vivo has been described. Adipose tissue represents an accessible source of ADSC, with similar characteristics to BMSC.METHODS: BMSCs were obtained from patients under-going total hip arthroplasty and ADSC from human adi-pose tissue obtained from lipectomy. Cells were grown in medium containing 15% human serum. Cultures were serum deprived for 2 d before cultivating under similar pro-hepatogenic conditions to those of liver develop-ment using a 2-step protocol with sequential addition of growth factors, cytokines and hormones. Hepatic differ-entiation was RT-PCR-assessed and liver-marker genes were immunohistochemically analysed. RESULTS: BMSC and ADSC exhibited a fibroblastic mor-phology that changed to a polygonal shape when cells differentiated. Expression of stem cell marker Thy1 de-creased in differentiated ADSC and BMSC. However, the expression of the hepatic markers, albumin and CYPs increased to a similar extent in differentiated BMSC and ADSC. Hepatic gene activation could be attributed to in-creased liver-enriched transcription factors (C/EBPβ and HNF4α), as demonstrated by adenoviral expression vec-tors.CONCLUSION: Mesenchymal stem cells can be induced to hepatogenic transdifferentiation in vitro. ADSCs have a similar hepatogenic differentiation potential to BMSC, but a longer culture period and higher proliferation ca-pacity. Therefore, adipose tissue may be an ideal source of large amounts of autologous stem cells, and may become an alternative for hepatocyte regeneration, liver cell transplantation or preclinical drug testing. | Raquel Taléns-Visconti Ana Bonora Ramiro Jover Vicente Mirabet Francisco Carbonell José Vicente Castell María José Gómez-Lechón | 2006 | World Journal of Gastroenterology2006，12，36： | 35 |
| 2 | Anti-fungal and anti-bacterial activities of ethanol extracts of selected traditional Chinese medicinal herbs显示文摘Objective:To evaluate in ritro antimicrobial activities of selected 58 ethno-medicinal plant extracts with a view to assess their therapeutic potential.Methods:A total of 58 traditional Chinese medicinal plants were carefully selected based on the literature review and their traditional use.The antimicrobial activities of ethanol extracts of these medicinal plants were tested against fungi(Aspergillus funigaius),yeast(Candida albicans),gram-negative(Acirelobacter haumannii and Pseudornnruis aeruginosa)and gram-positive bacteria(Staphglococcus aureus).The activities were tested at three different concentrations of 1.00,0.10 and 0.01 mg/mL.The data was analysed using Gene data Screener program.Results:The measured antimicrobial activities indicated that out of the 58 plant extracts,15 extracts showed anti-fungal activity and 23 extracts exhibited anti-bacterial activity.Eight plant extracts have exhibited both anti-bacterial and anti-fungal activities.For instance,Eucommia ulmoides,Pohgonum cuspidcrtum,Poria cocas and Uncaria rhineophylla showed activity against both bacterial and fungal strains,indicating their broad spectrum of activity.Conclusions:The results revealed that the ethanol extracts of 30 plants out of the selected 58 possess significant antimicrobial activities.It is interesting to note that the findings from the current study are consistent with the traditional use.A clear correlation has also been found between the antimicrobial activity and the flavonoid content of the plant extracts which is in agreement with the literature.Hence.the results presented here can be used to guide the selection of potential plant species for the isolation and structure elucidation of novel antimicrobial compounds in order to establish the structure-activity relationship.This in turn is expected to lead the way to the discovery of novel antimicrobial agents for therapeutic use. | Lin Zhang Anjaneya S.Ravipati Sundar R Koyyalamudi Sang Chul Jeong Narsimha Reddy John Bartlett Paul T.Smith Mercedes de la Cruz Maria Cndida Monteiro ngeles Melguizo Ester Jimnez Francisca Vicente | 2013 | Asian Pacific Journal of Tropical Medicine2013，6，9： | 23 |
| 3 | Role of IL-10 and TGF-β1 in local immunosuppression in HPV-associated cervical neoplasia显示文摘Cervical cancer is a worldwide disease that constitutes a significant public health problem, especially in developing countries, not only due to its high incidence but also because the most affected population comprises women who belong to marginalized socio-economic classes. Clinical and molecular research has identified immunological impairment in squamous intraepithelial cervical lesions and cervical cancer patients. Human Papillomavirus(HPV) has several mechanisms for avoiding the immune system: it down-regulates the expression of interferon and upregulates interleukin(IL)-10and transforming growth factor(TGF)-β1 to produce a local immunosuppressive environment, which, along with altered tumor surface antigens, forms an immunosuppressive network that inhibits the antitumor immune response. In this review we analyzed the available data on several deregulated cellular immune functions in patients with NIC Ⅰ, NIC Ⅱ and NIC Ⅲ and cervical cancer. The effects of immunosuppressive cytokines on innate immune response, T-cell activation and cellular factors that promote tumor cell proliferation in cervical cancer patients are summarized. We discuss the functional consequences of HPV E2, E6, and E7 protein interactions with IL-10 and TGF-β1 promoters in the induction of these cytokines and postulate its effect on the cellular immune response in squamous intraepithelial cervical lesions and cervical cancer patients. This review provides a comprehensive picture of the immunological functions of IL-10 and TGF-β1 in response to HPV in humans. | Kirvis Torres-Poveda Margarita Bahena-Román Claudia Madrid-González Ana I Burguete-García Víctor Hugo Bermúdez-Morales Oscar Peralta-Zaragoza Vicente Madrid-Marina | 2014 | World Journal of Clinical Oncology2014，5，4： | 21 |
| 4 | Factors predicting poor prognosis in ischemic colitis显示文摘瞄准:决定临床、分析、内视镜的因素与有关是化学家大肠炎(IC ) 严厉。方法:85 个病人的一个总数从 1996 年 1 月在回顾的研究被注册到 2004 年 5 月。有 53 女性和 32 男性(年龄 74.6+/-9.4 年,范围 45-89 年) 。病人作为 IC 被诊断。下列变量包括年龄,性别,从到承认的症状的外观的时间的经期,病历,药,凳子频率,临床的症状和症状被分析,验血(血克和基本生物化学的侧面) ,和内视镜的调查结果。病人们在温和 IC 组和严重 IC 组(外科或死亡) 被划分。质的变量用 chi 平方测试被分析,参量的数据用学生的 t (P<0.05 ) 被分析。结果:温和 IC 组 69 个病人被在于(42 女性和 27 男性,平均年龄 74.7+/-12.4 年) 。严重 IC 组由 16 个病人组成(11 女性和 5 男性,平均 73.8+/-12.4 年的年龄) 。因为麦克匪特斯氏疗法(没有外科) 的失败,一个病人死了, 15 个病人经历了外科(6 在内视镜的诊断以后并且 9 在 peroperatory 以后诊断) 。85 个病人(9.6%) 中的八个死了,其它被跟随在上面作为为 9.6+/-3.5 瞬间的门诊病人。人口统计的数据,病历,药和凳子频率在两个组(P>0.05 ) 是类似的。说正经的有病的病人比稍微有病的病人有更少的便血(37.5% 对 86.9% , P = 0.000 ) 。更多的心悸亢进(45.4% 对 10.1% , P = 0.011 ) 并且假腹膜炎的更高的流行签名(75% 对 5.7% , P = 0.000 ) 当腹的疼痛的存在和紧张在二个组之间是类似的时,在严重 IC 组被观察。当承认时,有严重 IC 的二个病人有吃惊。关于分析数据,更严重有病的病人被发现比略微有病的病人有贫血症和血钠过少(37.5% 对 10.1% , P = 0.014 和 46.6% 对 14.9% , P = 0.012,分别地) 。狭窄是那比在稍微有病的病人更经常出现在严重有病的病人的唯一的内视镜的发现(66.6% 对 17.3% , P = 0.017 ) 。结论:能预言 IC 的差的预后是便血,心悸亢进和假腹膜炎,贫血症和血钠过少和狭窄的缺席的因素。 | Ramón A■ón Marta Maia Boscá Vicente Sanchiz Joan Tosca Pedro Almela Cirilo Amorós Adolfo Benages | 2006 | World Journal of Gastroenterology2006，12，30： | 21 |
| 5 | 阻塞性睡眠呼吸暂停综合征男性患者持续正压通气治疗对心血管系统远期预后的疗效观察 | Marin J.M. Carrizo S.J. Vicente E. Agusti A.G.N. 黄卫东 | 2005 | 世界核心医学期刊文摘(神经病学分册)2005，0，9： | 21 |
| 6 | The Arabidopsis thaliana HAK5 K+ Transporter Is Required for Plant Growth and K+ Acquisition from Low K+ Solutions under Saline Conditions显示文摘在集中和它的部件的高亲密关系的范围的 K+ 举起广泛地被学习了。在 Arabidposis thaliana, AtHAK5 transporter 和 AtAKT1 隧道被显示了是涉及这个过程的主要运输蛋白质。这里,我们在二个重要压力条件下面学习这二个系统的角色:低 K+ 供应或咸度的存在。破坏 AtHAK5 和 AtAKT1 的 T-DNA 插入线为允许变异的线的生理的描述的长期的实验被采用。我们发现 AtHAK5 为必要的 K+ 吸收被要求在缺席以及面对咸度在低 K+ 支撑植物生长。咸度极大地减少了 AtHAK5 抄本层次并且支持了调停 AtAKT1 的 K+ 流出,导致 K+ 营养的一个重要缺陷。尽管有一个有限能力, AtHAK5 面对咸度从低 K+ 集中为 K+ 获得起一个主要作用。 | Manuel Nieves-Cordones Fernando Aleman Vicente Martinez Francisco Rubio | 2010 | Molecular Plant2010，3，2： | 17 |
| 7 | Failed medial patellofemoral ligament reconstruction:Causes and surgical strategies显示文摘Patel ar instability is a common clinical problem encountered by orthopedic surgeons specializing in the knee. For patients with chronic lateral patellar instability, the standard surgical approach is to stabilize the patella through a medial patellofemoral ligament(MPFL) reconstruction. Foreseeably, an increasing number of revision surgeries of the reconstructed MPFL will be seen in upcoming years. In this paper, the causes of failed MPFL reconstruction are analyzed:(1) incorrect surgical indication or inappropriate surgical technique/patient selection;(2) a technical error; and(3) an incorrect assessment of the concomitant risk factors for instability. An understanding of the anatomy and biomechanics of the MPFL and cautiousness with the imaging techniques while favoring clinical over radiological findings and the use of common sense to determine the adequate surgical technique for each particular case, are critical to minimizing MPFL surgery failure. Additionally, our approach to dealing with failure after primary MPFL reconstruction is also presented. | Vicente Sanchis-Alfonso Erik Montesinos-Berry Cristina Ramirez-Fuentes Joan Leal-Blanquet Pablo E Gelber Joan Carles Monllau | 2017 | World Journal of Orthopedics2017，8，2： | 14 |
| 8 | I.31, a new combination of probiotics, improves irritable bowel syndrome-related quality of life显示文摘AIM:To determine the dose-related effects of a novel probiotic combination,I.31,on irritable bowel syndrome(IBS)-related quality of life(IBS-QoL).METHODS:A multicenter,randomized,double-blind,placebo-controlled intervention clinical trial with three parallel arms was designed.A total of 84 patients(53female,31 male;age range 20-70 years)with IBS and diarrhea according to Rome-Ⅲcriteria were randomly allocated to receive one capsule a day for 6 wk containing:(1)I.31 high dose(n=28);(2)I.31 low dose(n=27);and(3)placebo(n=29).At baseline,and 3and 6 wk of treatment,patients filled the IBSQoL,Visceral Sensitivity Index(VSI),and global symptom relief questionnaires.RESULTS:During treatment,IBS-QoL increased in all groups,but this increment was significantly larger in patients treated with I.31 than in those receiving placebo(P=0.008).After 6 wk of treatment,IBS-QoL increased by 18±3 and 22±4 points in the high and the low dose groups,respectively(P=0.041 and P=0.023vs placebo),but only 9±3 in the placebo group.Gutspecific anxiety,as measured with VSI,also showed a significantly greater improvement after 6 wk of treatment in patients treated with probiotics(by 10±2 and14±2 points,high and low dose respectively,P<0.05for both vs 7±1 score increment in placebo).Symptom relief showed no significant changes between groups.No adverse drug reactions were reported following the consumption of probiotic or placebo capsules.CONCLUSION:A new combination of three different probiotic bacteria was superior to placebo in improving IBS-related quality of life in patients with IBS and diarrhea. | Vicente Lorenzo-Zú?iga Elba Llop Cristina Suárez Beatriz álvarez Luis Abreu Jordi Espadaler Jordi Serra | 2014 | World Journal of Gastroenterology2014，20，26： | 13 |
| 9 | Non invasive blood flow measurement in cerebellum detects minimal hepatic encephalopathy earlier than psychometric tests显示文摘AIM: To assess whether non invasive blood flow measurement by arterial spin labeling in several brain regions detects minimal hepatic encephalopathy. METHODS: Blood flow(BF) was analyzed by arterial spin labeling(ASL) in different brain areas of 14 controls, 24 cirrhotic patients without and 16 cirrhotic patients with minimal hepatic encephalopathy(MHE). Images were collected using a 3 Tesla MR scanner(Achieva 3T-TX, Philips, Netherlands). Pulsed ASL was performed. Patients showing MHE were detected using the battery Psychometric Hepatic Encephalopathy Score(PHES) consisting of five tests. Different cognitive and motor functions were also assessed: alterations in selective attention were evaluated using the Stroop test. Patients and controls also performed visuo-motor and bimanual coordination tests. Several biochemical parameters were measured: serum pro-inflammatory interleukins(IL-6 and IL-18), 3-nitrotyrosine, cGMP and nitrates+nitrites in plasma, and blood ammonia. Bivariate correlations were evaluated.RESULTS: In patients with MHE, BF was increased in cerebellar hemisphere(P = 0.03) and vermis(P = 0.012) and reduced in occipital lobe(P = 0.017). BF in cerebellar hemisphere was also increased in patients without MHE(P = 0.02). Bimanual coordination was impaired in patients without MHE(P = 0.05) and much more in patients with MHE(P < 0.0001). Visuo-motor coordination was impaired only in patients with MHE(P < 0.0001). Attention was slightly affected in patients without MHE and more strongly in patients with MHE(P< 0.0001). BF in cerebellar hemisphere and vermis correlated with performance in most tests of PHES [(number connection tests A(NCT-A), B(NCT-B)and line tracing test] and in the congruent task of Stroop test. BF in frontal lobe correlated with NCT-A. Performance in bimanual and visuomotor coordination tests correlated only with BF in cerebellar hemisphere. BF in occipital lobe correlates with performance in the PHES battery and with CFF. BF in cerebellar hemisphere correlates with plasma cGMP and nitric oxide(NO) metabolites. BF in vermis cerebellar also correlates with NO metabolites and with 3-nitrotyrosine. IL-18 in plasma correlates with BF in thalamus and occipital lobe.CONCLUSION: Non invasive BF determination in cerebellum using ASL may detect MHE earlier than the PHES. Altered NO-cGMP pathway seems to be associated to altered BF in cerebellum. | Vicente Felipo Amparo Urios Carla Giménez-Garzó Omar Cauli Maria-Jesús Andrés-Costa Olga González Miguel A Serra Javier Sánchez-González Roberto Aliaga Remedios Giner-Durán Vicente Belloch Carmina Montoliu | 2014 | World Journal of Gastroenterology2014，20，33： | 12 |
| 10 | Acute-on-Chronic Liver Failure Is a Distinct Syndrome That Develops in Patients With Acute Decompensation of Cirrhosis显示文摘 | Richard Moreau Rajiv Jalan Pere Gines Marco Pavesi Paolo Angeli Juan Cordoba Francois Durand Thierry Gustot Faouzi Saliba Marco Domenicali Alexander Gerbes Julia Wendon Carlo Alessandria Wim Laleman Stefan Zeuzem Jonel Trebicka Mauro Bernardi Vicente Arro | 2013 | Gastroenterology2013，，7： | 12 |
| 11 | The Measurement of Engagement and Burnout: A Two Sample Confirmatory Factor Analytic Approach显示文摘 | Wilmar B. Schaufeli Marisa Salanova Vicente González-romá Arnold B. Bakker | 2002 | Journal of Happiness Studies2002，，1： | 10 |
| 12 | Biodegradable stents in gastrointestinal endoscopy显示文摘Biodegradable stents(BDSs)are an attractive option to avoid ongoing dilation or surgery in patients with benign stenoses of the small and large intestines.The experience with the currently the only BDS for endoscopic placement,made of Poly-dioxanone,have shown promising results.However some aspects should be improved as are the fact that BDSs lose their radial force over time due to the degradable material,and that can cause stent-induced mucosal or parenchymal injury.This complication rate and modest clinical efficacy has to be carefully considered in individual patients prior to placement of BDSs.Otherwise,the price of these stents therefore it is nowadays an important limitation. | Vicente Lorenzo-Zú?iga Vicente Moreno-de-Vega Ingrid Marín Jaume Boix | 2014 | World Journal of Gastroenterology2014，20，9： | 10 |
| 13 | Occult hepatitis C virus infection: A new form of hepatitis C显示文摘Occult hepatitis C virus (HCV) infection is a new recently characterized entity. This occult infection can be present in two different clinical situations: in anti-HCV negative, serum HCV-RNA negative patients with abnormal liver function tests and in anti-HCV positive subjects with normal values of liver enzymes and without serum HCV- RNA. This review describes recent studies of occult HCV infection in both kinds of patients. | Vicente Carreo | 2006 | World Journal of Gastroenterology2006，12，43： | 10 |
| 14 | Endoscopic anti-reflux therapy for gastroesophageal reflux disease显示文摘Gastroesophageal reflux disease has an increasing incidence and prevalence worldwide.A significant proportion of patients have a suboptimal response to proton pump inhibitors or are unwilling to take lifelong medication due to concerns about long-term adverse effects.Endoscopic anti-reflux therapies offer a minimally invasive option for patients unwilling to undergo surgical treatment or take lifelong medication.The best candidates are those with a good response to proton pump inhibitors and without a significant sliding hiatal hernia.Transoral incisionless fundoplication and nonablative radiofrequency are the techniques with the largest body of evidence and that have been tested in several randomized clinical trials.Band-assisted ligation techniques,anti-reflux mucosectomy,antireflux mucosal ablation,and new plication devices have yielded promising results in recent noncontrolled studies.Nonetheless,the role of endoscopic procedures remains controversial due to limited long-term and comparative data,and no consensus exists in current clinical guidelines.This review provides an updated summary focused on the patient selection,technical details,clinical success,and safety of current and future endoscopic anti-reflux techniques. | Enrique Rodríguez de Santiago Eduardo Albéniz Fermin Estremera-Arevalo Carlos Teruel Sanchez-Vegazo Vicente Lorenzo-Zúñiga | 2021 | World Journal of Gastroenterology2021，27，39： | 9 |
| 15 | Diagnosis and treatment of pancreas divisum: A literature review显示文摘Background:Pancreas divisum is a congenital embryological disease caused by a lack of fusion between the ventral and dorsal pancreatic ducts in the early stages of embryogenesis.Recurrent acute pancreatitis,chronic pancreatitis or chronic abdominal pain are the main clinical syndromes at presentation and occur in only 5%of the patients with pancreas divisum.This review aimed to discuss diagnosis and treatment strategies in patients with symptomatic pancreas divisum.Data sources:We report a literature review from 1990 up to January 2018 to explore the various diagnostic modalities and surgical techniques and results reported in the surgical treatment of pancreas divisum.Results:There are limited reports available on this topic in the literature.We analyzed and described the main indications in the treatment of pancreas divisum,focusing on surgical treatment and a discussion of the different approaches.Furthermore,we report the results from our experience in two cases of pancreas divisum treated by pancreatic head resection with segmental duodenectomy(the Nakao procedure).Conclusions:Pancreas divisum is a common pancreatic malformation in which only a few patients develop a symptomatic disease.Surgical treatment is needed in case of endoscopic drainage failure and in cases complicated with chronic pancreatitis and local complications.Many techniques,of greater or lesser complexity,have been proposed. | Valentina Ferri Emilio Vicente Yolanda Quijano Benedetto Ielpo Hipolito Duran Eduardo Diaz Isabel Fabra Riccardo Caruso | 2019 | Hepatobiliary & Pancreatic Diseases International2019，18，4： | 9 |
| 16 | Prefoldins Negatively Regulate Cold Acclimation in Arabidopsis thaliana by Promoting Nuclear Proteasome-Mediated HY5 Degradation显示文摘冷环境适应的过程是从适度的区域的许多植物在暴露于低不会结冻的温度以后增加他们的结冰忍耐的重要适应回答。这回答的正确发展依靠调整寒冷应答的基因的表示模式的很多个抄写因素的合适的累积。多重研究揭示了涉及支持这些抄写因素的累积的许多分子的机制。然而,有趣地,机制在控制如此的累积保证他们的足够的层次仍然保持大部分未知含有。在这个工作,我们证明 prefoldins (PFD ) 控制 HY5 的层次,有在由激活 anthocyanin 生合成的冷环境适应的一个关键角色的一个 Arabidopsis 抄写因素,响应低温度。在他们与 HY5 交往的地方,我们的结果证明在冷条件下面, PFD 通过 DELLA 依赖的机制积累进原子核,触发它的 ubiquitination 和随后的降级。接着, HY5 的降级将导致 anthocyanin 生合成变细,保证冷环境适应的精确发展。这些调查结果揭开为在对不能生活的压力的工厂回答的 PFD 的未预见到的原子功能。 | Carlos Perea-Resa Miguel A. Rodriguez-Milla Elisa Iniesto Vicente Rubio Julio Salinas | 2017 | Molecular Plant2017，10，6： | 8 |
| 17 | Low-cost disordered carbons for Li/S batteries: A high- performance carbon with dual porosity derived from cherry pits显示文摘一微 -- 并且从樱桃坑废物获得并且与 H 3 PO 4 酸为 Li/S 电池作为硫主人被学习了。碳有 1,662 m 2 灀楡的一个高特定的表面区域吗? | Celia Hernandez-Rentero Rafael Cordoba Noelia Moreno Alvaro Caballero Julian Morales Mara Olivares-Marin Vicente Gomez-Serrano | 2018 | Nano Research2018，11，1： | 7 |
| 18 | Three-dimensional endoanal ultrasound for diagnosis of perianal fistulas: reliable and objective technique显示文摘AIM: To evaluate accuracy of three-dimensional endoanal ultrasound(3D-EAUS) as compared to 2D-EAUS and physical examination(PE) in diagnosis of perianal fistulas and correlate with intraoperative findings. METHODS: A prospective observational consecutive study was performed with patients included over a two years period. All patients were studied and operated on by the Colorectal Unit surgeons. The inclusion criteria were patients over 18, diagnosed with a criptoglandular perianal fistula. The PE, 2D-EAUS and 3D-EAUS was performed preoperatively by the same colorectal surgeon at the outpatient clinic prior to surgery and the fistula anatomy was defined and they were classified in intersphincteric, high or low transsphincteric, suprasphincteric and extrasphincteric. Special attention was paid to the presence of a secondary tract, the location of the internal opening(IO) and the site of external opening. The results of these different examinations were compared to the intraoperative findings. Data regarding location of the IO, primary tract, secondary tract, and the presence of abscesses or cavities wasanalysed.RESULTS: Seventy patients with a mean age of 47years(range 21-77), 51 male were included. Low transsphincteric fistulas were the most frequent type found(33, 47.1%) followed by high transsphincteric(24,34.3%) and intersphincteric fistulas(13, 18.6%). There are no significant differences between the number of IO diagnosed by the different techniques employed and surgery(P > 0.05) and, there is a good concordance between intraoperative findings and the 2D-EAUS(k= 0.67) and 3D-EAUS(k = 0.75) for the diagnosis of the primary tract. The ROC curves for the diagnosis of transsphincteric fistulas show that both ultrasound techniques are adequate for the diagnosis of low transsphincteric fistulas, 3D-EAUS is superior for the diagnosis of high transsphincteric fistulas and PE is weak for the diagnosis of both types.CONCLUSION: 3D-EAUS shows a higher accuracy than 2D-EAUS for assessing height of primary tract in transsphincteric fistulas. Both techniques show a good concordance with intraoperative finding for diagnosis of primary tracts. | Marina Garcés-Albir Stephanie Anne García-Botello Alejandro Espi Vicente Pla-Martí Jose Martin-Arevalo David Moro-Valdezate Joaquin Ortega | 2016 | World Journal of Gastrointestinal Surgery2016，8，7： | 7 |
| 19 | The Study of Carbamoyl Phosphate Synthetase 1 Deficiency Sheds Light on the Mechanism for Switching On/Off the Urea Cycle显示文摘Carbamoyl phosphate synthetase 1(CPS1) deficiency(CPS1D) is an inborn error of the urea cycle having autosomal(2q34) recessive inheritance that can cause hyperammonemia and neonatal death or mental retardation. We analyzed the effects on CPS1 activity, kinetic parameters and enzyme stability of missense mutations reported in patients with CPS1 deficiency that map in the 20-k Da C-terminal domain of the enzyme. This domain turns on or off the enzyme depending on whether the essential allosteric activator of CPS1, N-acetylL-glutamate(NAG), is bound or is not bound to it. To carry out the present studies, we exploited a novel system that allows the expression in vitro and the purification of human CPS1, thus permitting site-directed mutagenesis. These studies have clarified disease causation by individual mutations, identifying functionally important residues, and revealing that a number of mutations decrease the affinity of the enzyme for NAG. Patients with NAG affinity-decreasing mutations might benefit from NAG site saturation therapy with N-carbamyl-Lglutamate(a registered drug, the analog of NAG). Our results, together with additional present and prior site-directed mutagenesis data for other residues mapping in this domain, suggest an NAG-triggered conformational change in the b4-a4 loop of the C-terminal domain of this enzyme. This change might be an early event in the NAG activation process. Molecular dynamics simulations that were restrained according to the observed effects of the mutations are consistent with this hypothesis, providing further backing for this structurally plausible signaling mechanism by which NAG could trigger urea cycle activation via CPS1. | Carmen Díez-Fernández José Gallego Johannes H?berle Javier Cervera Vicente Rubio | 2015 | Journal of Genetics and Genomics2015，42，5： | 7 |
| 20 | Local recurrence of a parosteal osteosarcoma 21 years after incomplete resection显示文摘Parosteal osteosarcoma (POS) is the most common form of surface osteosarcoma. Its symptoms are insidious and its duration prior to diagnosis is considerably longer than that of other types of osteosarcoma. We report a case of POS with a growing mass but no evidence of metastasis. This tumor, which was diagnosed as calcified hematoma with benign characteristics, was incompletely resected in our hospital 21 years before the diagnosis of recurrence. The patient underwent a wide en bloc resection in our hospital and was free of symptoms, with no signs of tumor recurrence or metastasis during a 53-month follow-up. | Andrés Combalia Ernesto Muoz-Mahamud Antonio Palacín Jaume Pomés Vicente López | 2011 | Chinese Journal of Cancer2011，30，12： | 5 |
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