Targeted disruption of the mouse transforming growth factor-beta 1 gene results in multifocal inflammatory disease.

Transforming growth factor-beta 1 (TGF-beta 1) is a multifunctional growth factor that has profound regulatory effects on many developmental and physiological processes. Disruption of the TGF-beta 1 gene by homologous recombination in murine embryonic stem cells enables mice to be generated that carry the disrupted allele. Animals homozygous for the mutated TGF-beta 1 allele show no gross developmental abnormalities, but about 20 days after birth they succumb to a wasting syndrome accompanied by a multifocal, mixed inflammatory cell response and tissue necrosis, leading to organ failure and death. TGF-beta 1-deficient mice may be valuable models for human immune and inflammatory disorders, including autoimmune diseases, transplant rejection and graft versus host reactions.     

Simulation of Weld Depth in A-TIG Welding with Unified Arc-electrode model

Ｉｔｋｎｏｗｎｔｈａｔｓｍａｌｌａｍｏｕｎｔｓｏｆｆｌｕｘｏｎｔｈｅｓｕｒｆａｃｅｏｆｓｔａｉｎｌｅｓｓｓｔｅｅｌｃａｎｉｎｃｒｅａｓｅｔｈｅｄｅｐｔｈｏｆｗｅｌｄｐｅｎｅ ｔｒａｔｉｏｎｉｎＴＩＧｗｅｌｄｉｎｇｂｙａｆａｃｔｏｒｏｆｔｈｒｅｅ[1] .Ｔｈｉｓｐｒｏｃｅｓｓｉｓｒｅｆｅｒｒｅｄｔｏａｓ”ＡＴＩＧ”ｏｒＴＩＧｗｅｌｄｉｎｇａｃｔｉ ｖａｔｅｄｂｙｆｌｕｘ .Ｔｈｅｒｅｈａｖｅｂｅｅｎｔｈｒｅｅｐｕｂｌｉｓｈｅｄｐｈｙｓ ｉｃａｌｍｅｃｈａｎｉｓｍｓｔｈａｔａｒｅｐｏｓｓｉｂｌｅｃｏｎｔｒｉ…     

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.     

Mechanism for the learning deficits in a mouse model of neurofibromatosis type 1.

Neurofibromatosis type I (NF1) is one of the most common single-gene disorders that causes learning deficits in humans. Mice carrying a heterozygous null mutation of the Nfl gene (Nfl(+/-) show important features of the learning deficits associated with NF1 (ref. 2). Although neurofibromin has several known properties and functions, including Ras GTPase-activating protein activity, adenylyl cyclase modulation and microtubule binding, it is unclear which of these are essential for learning in mice and humans. Here we show that the learning deficits of Nf1(+/-) mice can be rescued by genetic and pharmacological manipulations that decrease Ras function. We also show that the Nf1(+/-) mice have increased GABA (gamma-amino butyric acid)-mediated inhibition and specific deficits in long-term potentiation, both of which can be reversed by decreasing Ras function. Our results indicate that the learning deficits associated with NF1 may be caused by excessive Ras activity, which leads to impairments in long-term potentiation caused by increased GABA-mediated inhibition. Our findings have implications for the development of treatments for learning deficits associated with NF1.     

The failing heart.

Cardiomyopathies are disorders affecting heart muscle that usually result in inadequate pumping of the heart. They are the most common cause of heart failure and each year kill more than 10,000 people in the United States. In recent years, there have been breakthroughs in understanding the molecular mechanisms involved in this group of conditions, with knowledge of the genetic basis for cardiomyopathies perhaps seeing the largest advance, enabling clinicians to devise improved diagnostic strategies and preparing the stage for new therapies.     

科技文献检索质量对科研工作的影响

本文对科技文献的作用，特征进行了分析。探讨了科技文献检索途径、方法及质量对科学研究活动的影响。     

PS4.0微机极谱法测定食品中微量钙

采用PS4.0微机极谱仪标准加入法测定了食品中的微量钙 ,采用DTD -40型恒温消解仪消化食品 ,检测下限 1 0 0 μg/L ,线性范围 0 .1 0～ 4.0 0mg/L ,相对标准偏差为 6.62 % ,回收率为97 2 % .锌、锰、铅、铜、镉不干扰测量 .分析结果令人满意     

Microbial degradation of bitumen

Summary A blown bitumen Mexphalte R 90/40 with a high content of saturated hydrocarbons was degraded by several microorganisms to the same extent. In batch cultures ofSaccharomycopsis lipolytica, maximal biodegradation was estimated to be about 9% w/w, 3.2·10^–3 g/cm² and 3.1·10^–3 cm of degraded bitumen. The Mexphalte R 90/40 degradation rate was closely coupled to biofilm formation. The microbial activity concerned predominantly the oxidation of saturated hydrocarbons. A direct distillation bitumen 80/100 with a low content of saturated hydrocarbons and a high content of aromatic hydrocarbons and resins was more resistant to biodegradation.     

Function of DnaJ and DnaK as chaperones in origin-specific DNA binding by RepA

Heat-shock proteins are normal constituents of cells whose synthesis is increased on exposure to various forms of stress. They are interesting because of their ubiquity and high conservation during evolution. Two families of heat-shock proteins, hsp60s and hsp70s, have been implicated in accelerating protein folding and oligomerization and also in maintaining proteins in an unfolded state, thus facilitating membrane transport. The Escherichia coli hsp70 analogue, DnaK, and two other heat-shock proteins, DnaJ and GrpE, are required for cell viability at high temperatures and are involved in DNA replication of phage lambda and plasmids P1 and F. These three proteins are involved in replication in vitro of P1 DNA along with many host replication proteins and the P1 RepA initiator protein. RepA exists in a stable protein complex with DnaJ containing a dimer each of RepA and DnaJ. We report here that DnaK and DnaJ mediate an alteration in the P1 initiator protein, rendering it much more active for oriP1 DNA binding.