Mutations in LMF1 cause combined lipase deficiency and severe hypertriglyceridemia

Hypertriglyceridemia is a hallmark of many disorders, including metabolic syndrome, diabetes, atherosclerosis and obesity. A well-known cause is the deficiency of lipoprotein lipase (LPL), a key enzyme in plasma triglyceride hydrolysis. Mice carrying the combined lipase deficiency (cld) mutation show severe hypertriglyceridemia owing to a decrease in the activity of LPL and a related enzyme, hepatic lipase (HL), caused by impaired maturation of nascent LPL and hepatic lipase polypeptides in the endoplasmic reticulum (ER). Here we identify the gene containing the cld mutation as Tmem112 and rename it Lmf1 (Lipase maturation factor 1). Lmf1 encodes a transmembrane protein with an evolutionarily conserved domain of unknown function that localizes to the ER. A human subject homozygous for a deleterious mutation in LMF1 also shows combined lipase deficiency with concomitant hypertriglyceridemia and associated disorders. Thus, through its profound effect on lipase activity, LMF1 emerges as an important candidate gene in hypertriglyceridemia.     

天然丝光沸石在甲醇氨化反应中的催化作用

研究了吉林省东辽县天然丝光沸石催化甲醇氨化反应的规律,考察了酸处理,反应温度,反应物进料比和接触时间对样品催化性能的影响。通过ＸＲＤ、ＮＨ３－ＴＰＤ,Ｐｙ（ＮＨ３）－ＩＲ等手段对催化剂的晶体结构和酸性进行了表征,得出了催化剂活性与其酸量存在对应关系的结论。     

蒎烷热裂解中反应规律的考察（Ⅰ）

考察了在空管（无催化剂）常压下，反应温度，载气（Ｎ２）流量发触时间等条件的变化对蒎烷转化率和二氢月桂烯选择性的影响。     

Ca1—nSrnS：Cu^＋,Eu^2＋的合成与光谱特征

研究了Ｃｕ＾＋，Ｅｕ＾２＋共激活的Ｃａ１－ｎＳｒｎＳ的发光性质，基质组分Ｃａ／Ｓｒ摩尔比与荧光性质的关系，Ｃｕ＾＋和Ｅｕ＾２＋在纯ＣａＳ基质中的发射光谱分别位于４３０ｎｍ及６３０ｎｍ附近，随着基质中Ｓｒ含量的增加，红、蓝发射带都向绿区移动；当基质为纯ＳｒＳ时，红、蓝发射带复合为一个主峰在５１７ｎｍ、半宽度为８０ｎｍ的带谱，实验结果表明：Ｃａ１－ｎＳｒｎＳ：Ｃｕ＾２＋，Ｅｕ＾２＋是一种红、蓝发射峰值     

对冰球运动员心理素质训练的研究

本文对我国优秀冰球运动员的心理素质进行了全面的调查和训练现场的技术指标统计分析,研究发现,运动员的心理素质是直接影响我国冰球运动水平提高的重要因素,通过对研究对象半年多的现场实验研究提出了心理素质训练的模式,以及在动运员身体训练、技术训练、战术训练中有效进行心理训练的方法。     

稀土氧化物及硫氧化物红色荧光粉的制备与发光性质研究

本文报道了铕激活的氧化钇、氧化镧、氧化钆、氧化镥及钇、镧、钆、镥的硫氧化物红色荧光粉的制备与其发光性质。实验证明以硫氧化钆为基质的荧光粉发光性能最佳,而硫氧化镧则为一种很有发展前途的基质。     

SHRIMP zircon U-Pb dating for volcanic rocks of the Dasi Formation in southeast Hubei Province, middle-lower reaches of the Yangtze River and its implications

The Jinniu Basin in southeast Hubei,located at the westernmost part of middle-lower val-ley of the Yangtze River,is one of the important vol-canic basins in East China. Volcanic rocks in the Jin-niu Basin are distributed mainly in the Majiashan Formation,the Lingxiang Formation and the Dasi Formation,consisting of rhyolite,basalt and basaltic andesite,(trachy)-basalt and basaltic trachy-andesite and (trachy)-andesite and (trachy)-dacite and rhyolite respectively,in which the Dasi volcanism is volumet-rically dominant and widespread. The Dasi volcanic rocks were selected for SHRIMP zircon U-Pb dating to confirm the timing of volcanism. The results indi-cate that there exist a large amount of magmatic zir-cons characterized by high U and Th contents in the volcanic rocks. The concordia ages for 13 points are 128 ± 1Ma (MSWD = 3.0). On account of the shape of zircons and Th/U ratios,this age is considered to represent the crystallization time of the Dasi volcan-ism. The volcanic rocks in the Dasi,Majiashan and Lingxiang Formations share similar trace elementand REE partition patterns as well as Sr-Nd isotopic compositions. In combination with the regional geol-ogy,it is proposed that the southeast Hubei volcanic rocks were formed mainly during the Early Creta-ceous,just like other volcanic basins in middle-lower Yangtze valley. A lithospheric extension is also sug-gested for tectonic regime in this region in the Cre-taceous Period.     

U-Pb dating of detrital zircons from the Wudangshan Group in the South Qinling and its geological significance

U-Pb dating was made by the LA-ICP-MS method for detrital zircons from the Wudangshan volcanic-sedimentary succession in the South Qinling. Samples comprise quartz sandstones of the Wudangshan Group collected from the base of the Yangping Formation and an upper layer of the Shuangtai Formation overlain its volcanic sequence, and two river-sand collections from the drainage systems cutting across the two formations, respectively. The results show that the Yangping detrital zircons are dominated by 830–780 Ma grains with a minor population of ~1.0–0.84 Ga and sporadic grains of ~2.6, ~2.4 and 2.0 Ga, whereas the Shuangtai zircons yield an upper intercept age of 763±33 Ma, identical to the timing of the Wudangshan volcanism within error, with few concordant grains of 1.9 and 0.86 Ga. Age spectra for the two river-sand samples are similar to those of the Yangping and Shuangtai Formations, respectively. It thus suggests that the Wudangshan strata are less than 780 Ma, whereas their major detrital zircon populations of 1.0–0.85 Ga and 830–780 Ma are consistent with timing of the Hannan magmatic activities along the northwestern margin of the South China Block. This suggests a Hannan or adjacent area provenance for the Wudangshan strata. The Wudang area is characterized by rift-related igneous events at ~755 and ~680 Ma, respectively, pointing to a tectono- magmatic history different from the Hannan area. It is inferred that the ~755 Ma magmatism is likely to indicate a separation of the South China Block from the supercontinent Rodinia, while the ~680 Ma event suggests a further split between the South Qinling and some unknown continent.     

Effects of typhoon Matsa on ionospheric TEC

The effects of typhoon Matsa on the ionosphere are studied by using GPS-TEC data observed at about 50 GPS stations.It is shown that the ionosphere has been already influenced and TEC tends to increase before the landing of Matsa,and the difference of TEC from its monthly median over the typhoon area is about 5 TECU.With the landing of Matsa,both the magnitude and the area of increased TEC decrease.One day after the landing of Matsa,TEC reaches its minimum and is lower than the monthly median.In comparison o...     

Preliminary study on climatic signals of stable isotopes from Holocene speleothems under monsoon condition

Within the East Asian monsoon zone the change in oxygen isotope of Holocene stalagmite is mainly related to monsoon rain rather than to temperature. And carbon isotope can be taken as a comprehensive signal which contains the natural information and the information of anthropogenic activity. Isotopic analysis is also compared with the annual layer records.