The gene encoding ganglioside-induced differentiation-associated protein 1 is mutated in axonal Charcot-Marie-Tooth type 4A disease.

We identified three distinct mutations and six mutant alleles in GDAP1 in three families with axonal Charcot-Marie-Tooth (CMT) neuropathy and vocal cord paresis, which were previously linked to the CMT4A locus on chromosome 8q21.1. These results establish the molecular etiology of CMT4A (MIM 214400) and suggest that it may be associated with both axonal and demyelinating phenotypes.     

体育教学的讲解与示范技巧

运用体育教育学的理论与方法,阐述体育教师在体育教学过程中的讲解艺术和示范技巧,并用常见的教学实例进行具体说明。     

我国税收收入超GDP增长的原因分析

文章依据我国近年来税收增长和GDP增长的统计数据，得出税收增长速度高于GDP增长速度的客观事实，并就其原因进行了综合分析，最后指出，从长远来看我国未来的税收增长必将回归到与经济同步增长或者略低于经济增长的轨道上来，以为政府决策提供参考。     

从图式理论看提高英语阅读能力的有效途径

教学实践说明,学好一门外语,有效的阅读是绝对必要的.然而笔者从期末教学测试的结果中发现,大多数学生的阅读理解题失分过多.……     

关中地区的贫水化探讨

关中地区水资源贫乏，供需矛盾尖锐，存在严重贫水化问题，这阻碍着陕西经济的进一步发展．本文主要论述了这一地区贫水化的表现，提出解决贫水化问题的措施和对策     

The first known use of vermillion

Vermillion has been shown to be useful in preserving human bones from 5000 years ago. Remarkably well-preserved human bones have been found in the dolmenic burial La Velilla in Osorno (Palencia, Spain), carefully covered by pulverized cinnabar (vermillion) which ensured their preservation even in non-favorable climatic conditions. We believe the red powder was deliberately deposited for preservative use because no cinnabar mine is to be found within 160 km, because of the large amount (hundreds of kilograms) used, and because its composition, red mercuric sulphide, is similar to that of preparations used in technical embalming. This finding pushes back the data of the use of mercury ore for preservation by four millennia in South America, and by at least one millennium in the Old World. Chemical and thermal analyses of vermillion in La Velilla have demonstrated its great purity and shown that the cinnabar was pulverized and washed (but not heated), producing a bright red-orange tone.     

反馈与启发式教学

当前学校教育面临加强培养创造型人才的严峻挑战,专家呼吁改变目前应试教育这种旧的模式是关键。     

结构疲劳寿命估算的计算机仿真

采用人工神经物方法描述了材料在疲劳损伤过程中的性能变化,将材料的疲劳过程看作一个离散事件,建立了既能反映累积损伤的非线性效应,又能反应映材料循环特性的疲劳损伤累积模型。     

High-precision timing of the Quaternary standard samples with thermal ionization mass spectrometry (TIMS) U-series method

Two national stalagmite standard (GBW04412, GBW04413) and one international coral standard (RKM_4) have been determined by using thermal ionization mass spectrometry (TIMS) method. The values of ( 234 U/ 238 U) act , ( 230 Th/ 234 U) act and age are all consistent in error range with the standard values obtained by using α spectrometry.     

用于瞳孔序列图像分析的实时轮廓检测和参数提取

本文介绍一种医用图像分析系统．该系统连续获取瞳孔图像,根据对图像序列的某些先验知识自动确定门限．在此基础上进行快速边缘检测,并自动修补缺损的目标轮廓．在判定所得轮廓有效后计算目标面积．若数据无效则自动重新采样．序列图像观测结束后对间隔不均匀的数据进行内插校正．系统所提供的分析结果可用于临床和医学研究