语言与社会：全球化的视野（下）

(上接 2 0 0 4年第 6期 )语言 ,族群和国家语言是识别自我认同感的一种重要手段 ,我们的认同意识与我们所说的语言有着密切的联系。正如我们所注意到的 ,语言是民族特征的主要标记之一 ,是创造族群意识的一种重要方法。1 8世纪末和 1 9世纪初 ,中欧的几个古老的国家逐渐发展成为工业化社会 ,学校教育也使越来越多的人具备了读写能力 ,当时使用曾一度被视作地方语言的人因此获得了经济和政治权力 ,他们开始使用自己的语言 ,以此作为民族特征的焦点。像匈牙利语、芬兰语、拉脱维亚语和波兰语等这样的语言的出现是和这些地区的民族意识的增强相…     

The Shwachman-Bodian-Diamond syndrome protein mediates translational activation of ribosomes in yeast

The autosomal recessive disorder Shwachman-Diamond syndrome, characterized by bone marrow failure and leukemia predisposition, is caused by deficiency of the highly conserved Shwachman-Bodian-Diamond syndrome (SBDS) protein. Here, we identify the function of the yeast SBDS ortholog Sdo1, showing that it is critical for the release and recycling of the nucleolar shuttling factor Tif6 from pre-60S ribosomes, a key step in 60S maturation and translational activation of ribosomes. Using genome-wide synthetic genetic array mapping, we identified multiple TIF6 gain-of-function alleles that suppressed the pre-60S nuclear export defects and cytoplasmic mislocalization of Tif6 observed in sdo1Delta cells. Sdo1 appears to function within a pathway containing elongation factor-like 1, and together they control translational activation of ribosomes. Thus, our data link defective late 60S ribosomal subunit maturation to an inherited bone marrow failure syndrome associated with leukemia predisposition.     

NIPBL, encoding a homolog of fungal Scc2-type sister chromatid cohesion proteins and fly Nipped-B, is mutated in Cornelia de Lange syndrome

Cornelia de Lange syndrome (CdLS) is a multiple malformation disorder characterized by dysmorphic facial features, mental retardation, growth delay and limb reduction defects. We indentified and characterized a new gene, NIPBL, that is mutated in individuals with CdLS and determined its structure and the structures of mouse, rat and zebrafish homologs. We named its protein product delangin. Vertebrate delangins have substantial homology to orthologs in flies, worms, plants and fungi, including Scc2-type sister chromatid cohesion proteins, and D. melanogaster Nipped-B. We propose that perturbed delangin function may inappropriately activate DLX genes, thereby contributing to the proximodistal limb patterning defects in CdLS. Genome analyses typically identify individual delangin or Nipped-B-like orthologs in diploid animal and plant genomes. The evolution of an ancestral sister chromatid cohesion protein to acquire an additional role in developmental gene regulation suggests that there are parallels between CdLS and Roberts syndrome.     

Embryonic megaloblastosis

Résumé La mégaloblastose embryonnaire peut apparaître à la suite d'un défaut métabolique dans la biosynthèse de novo des pyrimidines dans la conversion d'acide orotique à l'uridine 5-phosphate.     

上海市百岁老人的谱系及其分析

本文调查了上海市百岁老人的性别、职业和谱系,并绘制了57个完整的寿命谱系图,其中男性10名(占总数的17.5%),女性47名(占82.5%).男性中脑力劳动者4名(占男性总数的40%),体力劳动者6名(占60%);女性中脑力劳动者1人(占女性总数的2.1%),体力劳动者3人(占6.4%),操持家务者43人(占91.5%).有长寿家族史者40人(占寿命家族史清楚者的70.2%),无长寿家族史者17人(占29.8%).百岁老人配偶寿长≥70岁者,其已故子女的平均寿命高于配偶<70岁的.作者认为长寿是遗传的,并着重从遗传和环境两方面分析了女寿星数明显多于男寿星数的原因.     

心肌宁冲剂对抗垂体后叶素所致大鼠急性心肌缺血实验研究

心肌宁冲剂按1.75g/kg,0.875g/kg剂量,每天早晚分两次灌胃给药,连续7天,均能明显对抗垂体后叶素所诱发的大鼠急性心肌缺血的心电图改变。     

宇航员头部冲击的模拟创伤实验研究

该文通过３４只罗猴头颈实验研究，从中决定罗猴的颅脑损伤容限．当颅脑受到冲击时，颅壳发生局部变形瞬间，大脑亦发生变形，致使颅内压升高．利用这些结果来确定它的损伤机制，以模拟宇航员在冲击载荷下的创伤分析模型，将有助于进行临床创伤治疗和宇航弹射时的防护．