Hermansky-Pudlak syndrome type 7 (HPS-7) results from mutant dysbindin,a member of the biogenesis of lysosome-related organelles complex 1 (BLOC-1)

Hermansky-Pudlak syndrome (HPS; MIM 203300) is a genetically heterogeneous disorder characterized by oculocutaneous albinism, prolonged bleeding and pulmonary fibrosis due to abnormal vesicle trafficking to lysosomes and related organelles, such as melanosomes and platelet dense granules. In mice, at least 16 loci are associated with HPS, including sandy (sdy; ref. 7). Here we show that the sdy mutant mouse expresses no dysbindin protein owing to a deletion in the gene Dtnbp1 (encoding dysbindin) and that mutation of the human ortholog DTNBP1 causes a novel form of HPS called HPS-7. Dysbindin is a ubiquitously expressed protein that binds to alpha- and beta-dystrobrevins, components of the dystrophin-associated protein complex (DPC) in both muscle and nonmuscle cells. We also show that dysbindin is a component of the biogenesis of lysosome-related organelles complex 1 (BLOC-1; refs. 9-11), which regulates trafficking to lysosome-related organelles and includes the proteins pallidin, muted and cappuccino, which are associated with HPS in mice. These findings show that BLOC-1 is important in producing the HPS phenotype in humans, indicate that dysbindin has a role in the biogenesis of lysosome-related organelles and identify unexpected interactions between components of DPC and BLOC-1.     

Identification of Vangl2 and Scrb1 as planar polarity genes in mammals

In mammals, an example of planar cell polarity (PCP) is the uniform orientation of the hair cell stereociliary bundles within the cochlea. The PCP pathway of Drosophila refers to a conserved signalling pathway that regulates the coordinated orientation of cells or structures within the plane of an epithelium. Here we show that a mutation in Vangl2, a mammalian homologue of the Drosophila PCP gene Strabismus/Van Gogh, results in significant disruptions in the polarization of stereociliary bundles in mouse cochlea as a result of defects in the direction of movement and/or anchoring of the kinocilium within each hair cell. Similar, but less severe, defects are observed in animals containing a mutation in the LAP protein family gene Scrb1 (homologous with Drosophila scribble). Polarization defects in animals heterozygous for Vangl2 and Scrb1 are comparable with Vangl2 homozygotes, demonstrating genetic interactions between these genes in the regulation of PCP in mammals. These results demonstrate a role for the PCP pathway in planar polarization in mammals, and identify Scrb1 as a PCP gene.     

华南地区网纹甜瓜无土栽培技术

网纹甜瓜适宜干旱,雨水少,强光照,昼夜温差大的地区栽培,日本,澳州等国家采用温室栽培网纹甜瓜,其外观美,品质好。近年华南地区采用薄膜大棚作保护进行无土栽培网纹甜瓜,其外观和品质可与进口同类品种媲美。目前华南地区已成功引种日本网纹甜瓜,澳州网纹甜瓜和新疆哈蜜瓜、洋香瓜等,且大面积示范生产,并总结出一套较为成熟的技术,周年可安排种植三造,为拓展无土栽培新优品种,提高生产经济效益迈出可喜一步。 1.栽培方式 针对华南地区雨水多、热量足、昼夜温差小的气候特点,网纹甜瓜栽培,可选用薄膜大棚作保护,配     

信息熵通过系统变化规律的研究

对孤立热力系统中的不可逆过程，热力系统熵自发不可逆地增加；而对自发进行的物理变化过程，物理场熵是减小的。迄今，信息系统中信息熵的变化规律尚不十分明确。对此进行研究，得出结论：带限平稳正态随机信号通过因果最小相位线性系统后，熵可增加或减小，可用线性逆系统使熵可逆变化；平稳非正态独立同分布随机信号通过因果非最小相位线性系统后，熵恒增加，并趋近平稳正态信号，使熵趋于最大。原线性系统不能使熵可逆变化，亦不     

大型线性时不变奇摄动系统的燃料最优控制

讨论大型线性时不变奇摄动系统的燃料最优控制，证明这种系统的燃料最优控制问题可化为快，慢子系统的燃料最优控制来解决，并给出这种系统的近科燃料最优控制的设计方法。     

高尔基的早期创作

一八九八年春,两位当时无人知名的彼得堡出版商斯·多洛瓦陀大斯基和阿·查什尼可夫接连出版了两卷篇幅不大的高尔基作品集,集子用了一个极朴实的名字:《特写和短篇小说》。在决定出版之前,作者和出版商都颇为踌躇。但是书一出版,立即被读者抢购一空。收入这两卷的高尔基的二十篇作品,引起了广泛的注意。人们一再读它,一些著名评论家(尼·米哈依洛夫斯基、阿·波格丹诺维奇等)在大报刊上发表评论它的文章。高尔基成了全俄罗斯大名鼎鼎的人物。他的出版商开始筹备出版第三卷,同时准备再版头两卷。第二年秋,三卷都出版了,而且在几个星期内即告售罄。     

甘肃大蓟提取物对Hep细胞毒性作用研究

以Hep荷瘤小鼠为实验对象，给予不同剂量的甘肃大蓟提取物后，对瘤体、胸腺和脾脏重量以及对荷瘤小鼠免疫功能的影响进行测定分析。结果为，每天给予Hep荷瘤小鼠甘肃大蓟提取物10．0、20．0g／kg体质量（口服），连续给药10天后测定，与对熙组比，Hep细胞增殖抑制率为46．4％、59．7％，动物胸腺和脾脏的重量明显增加；组织病理学观察，给药组Hep瘤体均见坏死灶。坏死面积与给药剂量呈正相关。     

建筑企业施工合同的风险管理

介绍了施工企业合同风险的主要因素，评价了合同风险。从控制风险、转移风险、回避风险、抵御风险等方面论述了对合同风险的控制和管理。     

陶瓷膜处理油田采出水初探

油田采出水如直接排放，不仅污染环境，而且会造成大量水资源的浪费，直接回注则会引起一系列问题。陶瓷膜具有耐高温、机械强度高、耐酸碱性、使用寿命长等特点。采用陶瓷膜精细过滤处理油田采出水，可将其完全用于回注。着重讨论几种恢复陶瓷膜通量的方法，将其应用到油田采出水中，取得了较好的效果。     

出口贸易带动经济增长假设在中国的进一步实验

采用协整分析技术和Granger因果关系检验方法，检验了出口带动经济增长（Export-led growth,ELG）假设在中国的情况。结果表明，GDP、出口与贸易条件之间存在唯一协整关系，即三者之间存在长期的稳定均衡关系；存在出口增长到GDP增长的单向Granger因果关系，说明出口带动经济增长的假设在中国是成立的。