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| 1 | TALEN-based generation of a cynomolgus monkey disease model for human microcephaly显示文摘基因在非人类的首领编辑可以为在人探索病原学和遗传上基于的神经病学的混乱的治疗学的策略导致珍贵模型。然而,仔细在人模仿病理学、行为的赤字的神经病学的混乱的一个猴子模型成功地还没被产生了。Microcephalin 1 (MCPH1 ) 在人的大脑,和智力迟钝伴随的 MCPH1 变化原因头小畸型的进化被含有。这里,我们产生了用抄写带 biallelic MCPH1 变化的一只 cynomolgus 猴子(Macaca fascicularis ) 像使活跃之物的受动器核酸酶。猴子概括了大多数在病人观察的重要临床的特征,包括在头圆周,早熟的染色体冷凝作用(PCC ) ,语料库 callosum 的发育不全和上面的手足 spasticity 的显著减小。而且,在变异的真皮的成纤维细胞的 MCPH1 的 overexpression 救了 PCC 症候群。这个猴子模型可以帮助我们阐明在人的头小畸型并且更好的致病的 MCPH1 的角色在首领大脑尺寸的进化理解这蛋白质的功能。 | Qiong Ke Weiqiang Li Xingqiang Lai Hong Chen Lihua Huang Zhuang Kang Kai Li Jie Ren Xiaofeng Lin Haiqing Zheng Weijun Huang Yunhan Ma Dongdong Xu Zheng Chen Xinming Song Xinyi Lin Min Zhuang Tao Wang Fengfeng Zhuang Jianzhong Xi Frank Fuxiang Mao Huimin Xia Bruce T Lahn Qi Zhou Shihua Yang Andy Peng Xiang | 2016 | Cell Research2016，26，9： | 13 |
| 2 | Intrahepatic endometriosis as differential diagnosis: Case report and literature review显示文摘Intrahepatic endometriosis is one of the rarest forms of atypical endometriosis; only eighteen cases have been reported in the English literature. We describe the case of a 32-year-old woman, who presented with persistent, non-cyclical upper right quadrant abdominal pain, a central liver cyst, and no history of endometriosis. Three years previous, she was diagnosed with an intrahepatic cyst. The lesion progressed and two laparoscopic deroofing-operations were performed, yet the diagnosis of intrahepatic endometriosis was never reached. She presented in our clinic with further progress of the cyst as well as obstruction of the intrahepatic biliary system. The magnetic resonance imaging showed a 9.5 cm × 12 cm, lobulated intrahepatic cyst. We performed an ultrasonic pericystectomy. Immunostaining confirmed intrahepatic endometriosis. Only one of the previously described eighteen patients with intrahepatic endometriosis presented with cyclical pain in the upper right abdominal quadrant accompanying menstruation. This lack of a 'typical' clinic makes it challenging to diagnose extragonadal endometriosis without histopathology. A previous history of endometriosis was described in only twelve cases, thus the diagnosis of this condition should not be limited to patients with a known history of endometriosis. Six of 18 patients were postmenopausal, demonstrating this condition is not limited to women of reproductive age. A preoperative diagnosis was only reached in seven of the previously described cases, highlighting the importance of preoperative biopsies. Yet due to the potential adverse effects, a transhepatic biopsy must be discussed individually. Although rare, intrahepatic endometriosis should always be considered as a differential diagnosis in women with recurrent hepatic cysts, regardless of age or previous medical history. In such cases, histology is essential and a pericystectomy should be performed as standard of care. | Georg Fluegen Frank Jankowiak Luisa Zacarias Foehrding Feride Kroepil Wolfram T Knoefel Stefan A Topp | 2013 | World Journal of Gastroenterology2013，19，29： | 9 |
| 3 | 摄入玫瑰茄提取物后花青素-3-糖苷的药动学研究显示文摘 | Frank T 吴琳琳(摘) 黄正明(校) | 2006 | 国外医药(植物药分册)2006，21，1： | 6 |
| 4 | Cholangiocarcinoma, gone without the Wnt?显示文摘Cholangiocarcinoma(CCA) is a relatively rare malignancy of the intra- or extra-hepatic bile ducts that is classified according to its anatomical localization as intrahepatic, perihilar or distal. Overall, CCA has a dismal prognosis due to typical presentation at an advanced irresectable stage, lack of effective non-surgical treatments, and a high rate of disease recurrence. CCA frequently arises on a background of chronic liver inflammation and cholestasis. Chronic inflammation is accompanied by enhanced cell turnover with generation of additional inflammatory stimuli, and a microenvironment rich in pro-inflammatory mediators and proliferative factors that enable accumulation of mutations, transformation and expansion of mutated cells. A recent study by Boulter et al implicates the Wnt signaling cascade in cholangiocarcinogenesis. Wnt ligands Wnt7 B and Wnt10 A were found to be highly overexpressed in human CCA tissue. Wnt7 B protein was present throughout the tumor stroma, and often co-localized with a subset of CD68+ macrophages. To address in a direct manner whether Wnt signaling is engaged in development of CCA, Boulter et al explored the Wnt signaling pathway in an experimental model that recapitulates the multi-stage progression of human CCA.Wnt ligands found to be elevated in human CCA were also upregulated during the course of CCA development following thioacetamide treatment. Wnt10 a increased during the(pre-cancerous) regenerative phase, while Wnt7 b induction paralleled tumor growth. Along with upregulation of target genes, the findings demonstrate that the canonical Wnt pathway is progressively activated during cholangio-carcinogenesis. Macrophage depletion,eliminating a major source of Wnt7 b, prevented activation of the canonical Wnt cascade, and resulted in reduced number and volume of tumors in this model. Moreover,specific inhibitors of the canonical Wnt pathway(ICG-001 and C-59) caused reduction of tumor area and number,in xenograft and thioacetamide models of CCA. The aggregated findings show that experimental, and presumably human CCA, is a Wnt-driven tumor. Modulation of Wnt signaling, alone or in combination with surgicalor chemotherapy approaches, holds promise in the management of this fatal malignancy. | Anne T R Noll Thorsten Cramer Steven W M Olde Damink Frank G Schaap | 2016 | World Journal of Hepatology2016，8，26： | 3 |
| 5 | Endothelin-1 activation in pediatric patients undergoing surgical coarctation of the aorta repair显示文摘AIM To determine endothelin-1(ET-1)concentration before and after surgical coarctectomy and evaluate its association with left ventricular geometric change.METHODSA prospective,cohort study of 24 patients aged 2 d to 10years with coarctation of the aorta undergoing surgical repair.A sub-cohort of patients with age<1 mo was classified as'neonates'.Echocardiograms were performed just prior to surgery and in the immediate post-op period to assess left ventricle mass index and relative wall thickness(RWT).Plasma ET-1 levels were assessed at both time points.Association between ET-1 levels and ventricular remodeling was assessed.RESULTSPatients<1 year demonstrated higher pre-op ET-1 than post-op(2.8 pg/m L vs 1.9 pg/m L,P=0.02).Conversely,patients>1 year had no change in ET-1 concentration before and after surgery(1.1 vs 1.4,NS).Pre-op,patients<1 year demonstrated significantly higher ET-1 than older children(2.8 vs 1.1,P=0.001).Post-op there was no difference between the age groups(1.9 vs 1.4,NS).Neither RWT nor left ventricle mass index(LVMI)varied from pre-op to post-op.The subset of neonates showed a strong positive correlation between pre-op ET-1 and RWT(r=0.92,P=0.001).Patients with ET-1>2 pg/m L preop demonstrated higher LVMI(65.7 g/m^(2.7) vs 38.5 g/m^(2.7),P=0.004)and a trend towards higher RWT(45%vs39%,P=0.07)prior to repair than those with lower ET-1concentration.CONCLUSIONET-1 concentration is significantly variable in the perioperative period surrounding coarctectomy.Older children and infants have different responses to surgical repair suggesting different mechanisms of activation. | Benjamin Steven Frank Tracy T Urban Suhong Tong Courtney Cassidy Max B Mitchell Christopher S Nichols Jesse A Davidson | 2017 | World Journal of Cardiology2017，9，12： | 2 |
| 6 | The herpes simplex virus proeapsid: structure, conformational changes upon maturation, and roles of the triplex proteins VP19c and VP23 in assembly显示文摘 | Benes L T Frank P B William W | 1996 | J MoL BioL1996，263，： | 1 |
| 7 | Neural stem cells as a novel platform for tumor-specific delivery of therapeutic antibodies显示文摘 | FRANK R T EDMISTON M KENDALL S E | 2009 | PLoS One2009，4，12： | 1 |
| 8 | Advanced glyeation end-products induce cell cycle arrest and hypertrophy in podocytes 显示文摘 | Ruster C Bondeva T Franke S | 2008 | Nephrol Dial Transplant2008，23，： | 1 |
| 9 | Cuticularpermeance in relation to wax and cutin development along thegrowing barley(Hordeum vulgare)leaf显示文摘 | Richardson A Wojciechowski T Franke R | 2007 | Planta2007，225，6： | 1 |
| 10 | New constraints on the sources and behavior of neodymium and hafnium in seawater from Pacific Ocean ferromanganese crusts显示文摘 | VAN DE FLIERDT T FRANK M LEE D C | 2004 | Geochim et Cosmochim Acta2004，68，19： | 1 |
| 11 | Medication errors in United States Hospitals 显示文摘 | Bond CA Raehl CL Franke T | 2001 | Pharmacotherapy2001，21，9： | 1 |
| 12 | Interrelationships among mortality rates, drug costs, total cost of care, and length of stay in United States hospitals: summary and recom- mendations for clinical pharmacy services and staffing显示文摘 | Bond CA Raehl CL Franke T | 2001 | Pharmacotherapy2001，21，2： | 1 |
| 13 | Colorectal cancer stem cells: biology and therapeutic implications显示文摘 | Wilson BJ Schatton T Frank MH | 2011 | Curr Colorectal Cancer Rep2011，7，2： | 1 |
| 14 | Catching up and Convergence: Long- run Growth in East Asia 显示文摘 | Hsiao Frank S T Hsiao Mei - Chu W | 2004 | Review of Development Economics2004，8，2： | 1 |
| 15 | Advanced glycation end- products induce cell cycle arrest and hypertrophy in podocytes 显示文摘 | Rtister C Bondeva T Franke S | 2008 | Nephrol Dial Transplant2008，23，7： | 1 |
| 16 | Measurements and correlations of the physical of CO_Wheavy crude oil mixtures显示文摘 | Chung Frank T H Jones Ray A Nguyen Hai T | 1988 | SPE Reservoir Engi- neering1988，40，8： | 1 |
| 17 | Effects of individual climatic parameters on the infrared thermography of buildings 显示文摘 | Lehmann B Ghazi Wakili K Frank T | 2013 | Applied Energy2013，110，： | 1 |
| 18 | Linking objec- tively measured physical activity with objectively meas- ured urban form--findings from SMARTRAQ显示文摘 | Frank L D Schmid T L Sallis J F | 2005 | Amer- ican Journal of Preventive Medicine2005，28，22： | 1 |
| 19 | A Need-hierarchy Framework for Corporate Social Responsi- bility显示文摘 | Frank T | 1981 | The Academy of Management Review1981，，1： | 1 |
| 20 | Predictors of improved renal function after percutaneous stent-supported angioplasty of severe atherosclerotic ostial renal artery stenosis显示文摘 | Frank U Müller C | 2003 | Circulation2003，108，： | 1 |
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