日语的人文特征在语言意识方面的折射

本文主要从日本的历史,自然环境等方面,对日本语言发展、文学意识流、言语特征及使用心理状态方面的影响进行了探讨。     

腐植酸中官能团的测定方法的研究

研究了腐植酸中总酸性基、羧基、羰基、醌基、甲氧基等基团的测定方法．     

A mouse model for spinal muscular atrophy

The survival motor neuron gene is present in humans in a telomeric copy, SMN1, and several centromeric copies, SMN2. Homozygous mutation of SMN1 is associated with proximal spinal muscular atrophy (SMA), a severe motor neuron disease characterized by early childhood onset of progressive muscle weakness. To understand the functional role of SMN1 in SMA, we produced mouse lines deficient for mouse Smn and transgenic mouse lines that expressed human SMN2. Smn-/- mice died during the peri-implantation stage. In contrast, transgenic mice harbouring SMN2 in the Smn-/- background showed pathological changes in the spinal cord and skeletal muscles similar to those of SMA patients. The severity of the pathological changes in these mice correlated with the amount of SMN protein that contained the region encoded by exon 7. Our results demonstrate that SMN2 can partially compensate for lack of SMN1. The variable phenotypes of Smn-/-SMN2 mice reflect those seen in SMA patients, providing a mouse model for this disease.     

Msx2 deficiency in mice causes pleiotropic defects in bone growth and ectodermal organ formation

The composite structure of the mammalian skull, which forms predominantly via intramembranous ossification, requires precise pre- and post-natal growth regulation of individual calvarial elements. Disturbances of this process frequently cause severe clinical manifestations in humans. Enhanced DNA binding by a mutant MSX2 homeodomain results in a gain of function and produces craniosynostosis in humans. Here we show that Msx2-deficient mice have defects of skull ossification and persistent calvarial foramen. This phenotype results from defective proliferation of osteoprogenitors at the osteogenic front during calvarial morphogenesis, and closely resembles that associated with human MSX2 haploinsufficiency in parietal foramina (PFM). Msx2-/- mice also have defects in endochondral bone formation. In the axial and appendicular skeleton, post-natal deficits in Pth/Pthrp receptor (Pthr) signalling and in expression of marker genes for bone differentiation indicate that Msx2 is required for both chondrogenesis and osteogenesis. Consistent with phenotypes associated with PFM, Msx2-mutant mice also display defective tooth, hair follicle and mammary gland development, and seizures, the latter accompanied by abnormal development of the cerebellum. Most Msx2-mutant phenotypes, including calvarial defects, are enhanced by genetic combination with Msx1 loss of function, indicating that Msx gene dosage can modify expression of the PFM phenotype. Our results provide a developmental basis for PFM and demonstrate that Msx2 is essential at multiple sites during organogenesis.     

Functional haploinsufficiency of the human homeobox gene MSX2 causes defects in skull ossification

The genetic analysis of congenital skull malformations provides insight into normal mechanisms of calvarial osteogenesis. Enlarged parietal foramina (PFM) are oval defects of the parietal bones caused by deficient ossification around the parietal notch, which is normally obliterated during the fifth fetal month. PFM are usually asymptomatic, but may be associated with headache, scalp defects and structural or vascular malformations of the brain. Inheritance is frequently autosomal dominant, but no causative mutations have been identified in non-syndromic cases. We describe here heterozygous mutations of the homeobox gene MSX2 (located on 5q34-q35) in three unrelated families with PFM. One is a deletion of approximately 206 kb including the entire gene and the others are intragenic mutations of the DNA-binding homeodomain (RK159-160del and R172H) that predict disruption of critical intramolecular and DNA contacts. Mouse Msx2 protein with either of the homeodomain mutations exhibited more than 85% reduction in binding to an optimal Msx2 DNA-binding site. Our findings contrast with the only described MSX2 homeodomain mutation (P148H), associated with craniosynostosis, that binds with enhanced affinity to the same target. This demonstrates that MSX2 dosage is critical for human skull development and suggests that PFM and craniosynostosis result, respectively, from loss and gain of activity in an MSX2-mediated pathway of calvarial osteogenic differentiation.     

Structure and Properties of Porous Silk Fibroin Materials

In this article the structure and properties of freeze-dried porous silk fibroin materials were tested and analyzed.The results indicated that for porous silk fibroin materials prepared by means of freeze-drying,if freez-ing temperature was below —20℃,the structure of silk fibroin was mainly amorphous with a little silk Ⅱ crystal structure,and if freezing temperature was above —20℃,quite a lot of silk Ⅰ crystal structure formed.Porous silk fibroin materials,with average pore diameter be-     

吸收比-系数倍率法及其应用——水杨酸钠、安替比林和咖啡因的同时测定

用吸收比－系数倍率法同时测定撒痛风注射液中水杨酸钠、安替比林和咖啡因三组分的含量。它们的回收率分别为１００．７％～１０２．１％、９５．９３％～１０４．５％和９７．５７％～１０１．７％。     

煤炭工业结构优化的战略思考

以系统的观点探讨了我国煤炭工业结构优化的内涵及特征,论述了煤炭工业结构优化的必要性,进而提出了煤炭工业结构优化的目标及实现的主要措施。     

酶促半合成人胰岛素的副反应及纯化

研究酶促半合成人胰岛素的副反应．采用DEAE－SephadexA－25阴离子交换层析纯化合成人胰岛素中间体衍生物,去除脱八肽胰岛素以及人胰岛素类似物,精制酶促半合成人胰岛素．