Ru2 and Ru encode mouse orthologs of the genes mutated in human Hermansky-Pudlak syndrome types 5 and 6

Hermansky-Pudlak syndrome (HPS) is a genetically heterogeneous disease involving abnormalities of melanosomes, platelet dense granules and lysosomes. Here we have used positional candidate and transgenic rescue approaches to identify the genes mutated in ruby-eye 2 and ruby-eye mice (ru2 and ru, respectively), two 'mimic' mouse models of HPS. We also show that these genes are orthologs of the genes mutated in individuals with HPS types 5 and 6, respectively, and that their protein products directly interact. Both genes are previously unknown and are found only in higher eukaryotes, and together represent a new class of genes that have evolved in higher organisms to govern the synthesis of highly specialized lysosome-related organelles.     

A defective response to Hedgehog signaling in disorders of cholesterol biosynthesis

Smith-Lemli-Opitz syndrome (SLOS), desmosterolosis and lathosterolosis are human syndromes caused by defects in the final stages of cholesterol biosynthesis. Many of the developmental malformations in these syndromes occur in tissues and structures whose embryonic patterning depends on signaling by the Hedgehog (Hh) family of secreted proteins. Here we report that response to the Hh signal is compromised in mutant cells from mouse models of SLOS and lathosterolosis and in normal cells pharmacologically depleted of sterols. We show that decreasing levels of cellular sterols correlate with diminishing responsiveness to the Hh signal. This diminished response occurs at sterol levels sufficient for normal autoprocessing of Hh protein, which requires cholesterol as cofactor and covalent adduct. We further find that sterol depletion affects the activity of Smoothened (Smo), an essential component of the Hh signal transduction apparatus.     

Language-tree divergence times support the Anatolian theory of Indo-European origin

Languages, like genes, provide vital clues about human history. The origin of the Indo-European language family is "the most intensively studied, yet still most recalcitrant, problem of historical linguistics". Numerous genetic studies of Indo-European origins have also produced inconclusive results. Here we analyse linguistic data using computational methods derived from evolutionary biology. We test two theories of Indo-European origin: the 'Kurgan expansion' and the 'Anatolian farming' hypotheses. The Kurgan theory centres on possible archaeological evidence for an expansion into Europe and the Near East by Kurgan horsemen beginning in the sixth millennium BP. In contrast, the Anatolian theory claims that Indo-European languages expanded with the spread of agriculture from Anatolia around 8,000-9,500 years bp. In striking agreement with the Anatolian hypothesis, our analysis of a matrix of 87 languages with 2,449 lexical items produced an estimated age range for the initial Indo-European divergence of between 7,800 and 9,800 years bp. These results were robust to changes in coding procedures, calibration points, rooting of the trees and priors in the bayesian analysis.     

The extent and patterns of usage of Agent Orange and other herbicides in Vietnam

Herbicides including Agent Orange were sprayed by United States forces for military purposes during the Vietnam War (1961-1971) at a rate more than an order of magnitude greater than for similar domestic weed control. In 1974, the US National Academy of Sciences published estimates of the extent and distribution of herbicides sprayed. Here we present revised estimates, developed using more-complete data. The spray inventory is expanded by more than seven million litres, in particular with heavily dioxin-contaminated herbicides. Estimates for the amount of dioxin sprayed are almost doubled. Hamlet census data reveal that millions of Vietnamese were likely to have been sprayed upon directly. Our identification of specific military herbicide targets has led to a more coherent understanding of spraying. Common errors in earlier interpretations of the spray data are also discussed.     

中美科学合作:文献计量学分析

利用SCI论文中作者间的国际合作信息,分析了纳米科技、化学、遗传学和细胞生物学领域44个研究方向中国和美国在1996年至2005年10年间的科学合作发展态势.从中美合作论文的数量增长、合作强度的趋强、中国和美国各自最重要的合作伙伴以及与科技发达国家的国际合作等方面证明中美在重要研究方向上的合作发展迅速,成效显著.结果表明,中美合作之所以发展迅速,在美国的中国学者和华人发挥了重要作用.     

Stoneflies (Plecoptera) of Mount Rainier National Park, Washington

Mount Rainier National Park, with an area of 95,356 ha, is approximately one-third as large as the state of Rhode Island. The lowest point is 490 m in elevation in the southeastern corner near where the Ohanapecosh River crosses the southern boundary. Columbia Crest is the highest point at 4392 m. The entire park is a rugged landscape marked by the major topographical feature, Mount Rainier, comprising over 25,899 ha, almost one-third of the park. The park lies entirely west of the crest line of the Cascade Range. Most streams in the park originate on Mount Rainier; however, several large rivers meander through the park near its boundaries. One of the first attempts to summarize the stoneflies of Washington, including Mount Rainier National Park, was Hoppe's 1938 work that reported ca 8 species. Jewett (1959) reviewed the stoneflies of the Pacific Northwest and listed 7 species that had type localities in the park: Megaleuctra kincaidi Frison, Doddsia occidentalis (Banks), Soliperla fenderi ( Jewett), Frisonia picticeps (Hanson), Isoperla rainiera Jewett, Megarcys irregularis (Banks), and M. subtruncata (Hanson). Subsequently, Kathroperla takhoma Stark and Surdick (1987) was described from the park. Samples of adult stoneflies from 1994 to 2001 indicate the presence of at least 82 species, with 64% of these typical Pacific Northwest species, and 30 species, or 36%, widespread western North American species. Seventeen new Washington state records are listed, including a substantial range extension for Lednia tumana (Ricker). One undescribed species in the Sweltsa borealis complex was also discovered. We also present illustrations of male terminalia for Despaxia augusta (Banks) and Moselia infuscata (Claassen) to aid in the identification of these species.     

Note on mound architecture of the black-tailed praire dog

The development of black-tailed prairie dog ( Cynomys ludovicianus ) burrow mounds was monitored for three years. Entrances were flagged at points where a prairie dog was observed digging into the ground (primary entrances, n = 22) and digging out of the ground (secondary entrances, n = 8). In all samples it was observed that primary entrances became dome mounds and secondary entrances crater mounds. It is suggested that, although induced airflow (presently, a popular model) may partly explain the presence of mounds, architectural types (dome vs. crater mounds) are the result of energy constraints associated with building materials that differ at the entrances.