| 5 | Age-related Changes in Familial Hypertrophic Cardiomyopathy Phenotype in Transgenic Mice and Humans显示文摘β-myosin heavy chain mutations are the most frequently identified basis for hypertrophic cardiomyopathy(HCM). A transgenic mouse model(αMHC403) has been extensively used to study various mechanistic aspects of HCM. There is general skepticism whether mouse and human disease features are similar. Herein we compare morphologic and functional characteristics, and disease evolution, in a transgenic mouse and a single family with a MHC mutation. Ten male αMHC403 transgenic mice(at –5 weeks, –12 weeks, and –24 weeks) and 10 HCM patients from the same family with a β–myosin heavy chain mutation were enrolled. Morphometric, conventional echocardiographic, tissue Doppler and strain analytic characteristics of transgenic mice and HCM patients were assessed. Ten male transgenic mice(αMHC403) were examined at ages –5 weeks, –12 weeks, and –24 weeks. In the transgenic mice, aging was associated with a significant increase in septal(0.59±0.06 vs. 0.64±0.05 vs. 0.69±0.11 mm, P<0.01) and anterior wall thickness(0.58±0.1 vs. 0.62±0.07 vs. 0.80±0.16 mm, P<0.001), which was coincident with a significant decrease in circumferential strain(–22%±4% vs. –20%±3% vs. –19%±3%, P=0.03), global longitudinal strain(–19%±3% vs. –17%±2% vs. –16%±3%, P=0.001) and E/A ratio(1.9±0.3 vs. 1.7±0.3 vs. 1.4±0.3, P=0.01). The HCM patients were classified into 1st generation(n=6; mean age 53±6 years), and 2nd generation(n=4; mean age 32±8 years). Septal thickness(2.2±0.9 vs. 1.4±0.1 cm, P<0.05), left atrial(LA) volume(62±16 vs. 41±5 mL, P=0.03), E/A ratio(0.77±0.21 vs. 1.1±0.1, P=0.01), E/e' ratio(25±10 vs. 12±2, P=0.03), global left ventricular(LV) strain(–14%±3% vs. –20%±3%, P=0.01) and global LV early diastolic strain rate(0.76±0.17 s-1 vs. 1.3±0.2 s-1, P=0.01) were significantly worse in the older generation. In β-myosin heavy chain mutations, transgenic mice and humans have similar progression in morphologic and functional abnormalities. The αMHC403 transgenic mouse model closely recapitulates human disease. | 罗鸿昌 Iraklis Pozios Styliani Vakrou Lars Sorensen Roselle M.Abraham Theodore Abraham | 2014 | Journal of Huazhong University of Science and Technology(Medical Sciences)2014，34，5： | 1 |
| 7 | 顺铂化疗对口腔颌面部鳞癌细胞基因表达谱的影响显示文摘目的探讨口腔颌面部鳞癌对顺铂的应答规律,以阐明肿瘤耐药的作用机制,指导临床化疗。方法收集(8例)4对临床标本,根据其是否接受过顺铂化疗,分为2组,采用AffymetrixHG-U95Av2寡核苷酸芯片对其基因表达谱进行聚类分析。结果应用Genespring软件同时对标本和基因进行聚类,发现根据标本是否接受过顺铂化疗及化疗效果可正确聚类,而且利用基因聚类的结果,筛选出关系最密切的50条基因,其表达情况能够正确预测标本是否接受过顺铂化疗。结论顺铂化疗可改变一组基因在口腔鳞癌的表达,可以通过该组基因的表达谱对标本进行正确聚类,而且这种改变可能与口腔鳞癌顺铂化疗效果相关。 | 张萍 陈万涛 徐骎 陈方安 M.Abraham Kuriakose 邱蔚六 | 2003 | 中国口腔颌面外科杂志2003，1，3： | 0 |
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