Mitochondrial diseases is one of the common genetic metabolic diseases in children.The inheritance patterns are complex,both mendelian inheritance and mitochondrial maternal inheritance.Although the clinical feature is various and the diagnosis is difficult,but with the development of molecular biology,some clinical and genetic features in mitochondrial syndrome were gradually clear.This paper introduced the clinical feature and genetic characteristics in several common mitochondrial syndrome of children.

After the difficult position of hospital library and the experiences of Shanghai Public Health Clinical Center Library in meeting clinical and medical needs were described, such as relying on the advantages of Fudan library col-league, improving professional literacy, providing subject service, and expanding service contents, the author pointed out that providing excellent service through multiple channels and ways is the key for hospital library to improve its value and position and its sustainable development .

Using brartemicin as the leading compound, fifteen novel trehalose derivatives were designed and synthesized, and the structures were determined by 11H NMR, MS and element analysis. Inhibitory effects of the target compounds on the proliferation of A549, HepG2 and HUVEC cells were detectec by MTT assay. The abilities of adhesion, invasion and migration of A549 and HepG2 cells inhibited by the synthesized compounds were evaluated through Matrigel experiment and Transwell assay. The results showed that, the target compounds had no significant cytotoxicity (compared with the control, P>0.05) to A549, HepG2 and HUVEC cells at the dose range of 1-32 µmol.L-1. At the above dose range, the inhibitory effects of A549 cells adhesion, invasion and migration and HepG2 cells adhesion and invasion by compounds 79 and 82 are better than brartemicin.
Antineoplastic Agents ; chemical synthesis ; pharmacology ; Cell Adhesion ; Cell Line, Tumor ; drug effects ; Drug Design ; Hep G2 Cells ; drug effects ; Human Umbilical Vein Endothelial Cells ; drug effects ; Humans ; Trehalose ; analogs & derivatives ; chemistry

Autistic Disorder ; immunology ; physiopathology ; Humans ; Immune System ; physiopathology

Objective To investigate the clinical significance of serum interferon-λ1(IFN-λ1) levels in patients with systemic lupus erythematosus(SLE).Methods ELBA was used to evaluate the serum level of interferon-λ1 in 60 patients with SLE and 20 healthy individuals as normal control.SPSS10.0 statistic software was employed to investigate the relation between the serum IFN-λ1 level,the laboratory index and SLE disease activity index(SLEDAI).Results The serum IFN-λ1 level in SLE patients(74.2±16.1 pg/ml) was higher than that of the normal controls(70.4±12.6 pg/ml,P=0.339).The serum IFN-λ1 level in SLE patients with leucopenia(80.1±18.1 pg/ml) was higher than those of the normal control(P=0.052) and the SLE patients without leucopenia(70.6±13.7 pg/ml,P=0.024).The serum IFN-λ1 level was not correlated with SLEDAI,complements C3,C4,24-hour-proteinuria.Conclusion IFN-λ1 may be involved in the pathogenesis of leukopenia in SLE and it has not been proved that its level was correlated with the disease activity.

There are about 170 kinds of medical plants of Commelina all over the world.Experimental studies showed that the main chemical compositions in the plants included alkaloids,flavonoids,terpenes,and sterols etc.Plants of commelina,especially commelina communis,have pharmacological activities of antibacterial,antiinflammation,resisting-oxidation,lowering-glycemia,relieving-pain and stopping-cough etc.It is necessary that their pharmacological effects and chemical compositions are further developed and investigated systematically.

Peroxisome proliferator-activated receptors(PPAR) are ligand-activated nuclear transcription factors which regulate the expression of many genes.Numbers of researchs indicate PPAR exert anti-inflammatory activities by inhibiting the expression of inflammatory singal transcription passage such as NF-?B,AP-1,STAT,meanwhile preserve from acute lung injury.This review will focus on structure of PPAR and their ligand and activity regulation and antiinflammation mechanism of action and protection to acute lung injury.

Objective To establish the retrovirus expression vector containing glucose transporter-2 ( GLUT-2) gene and its stable virus producing cell line,for the construction of new "artificial islet beta cells". Methods Plasmid pCB7 /GLUT2 was digested by EcoRⅠ/BamHⅠ and cloned to retroviral vector PLXSN for the construction of recombinant plasmid pL-GLUT2-SN,which was identified by enzyme digestion and sequencing,and then transfected to packaging cell line PA317. Titer of the recombinant virus was detected. Cell lines with a high titer were cloned and identified by PCR and RT-PCR. Results The retrovirus expression vector pL-GLUT2-SN was established,in which the insertion site and reading frame of target gene were confirmed by enzyme digestion and sequence analysis. The highest titer of virus producing cell line PA317 /GLUT2 was 7. 1 ? 105 CFU/mL. PCR and RT-PCR showed that the GLUT-2 gene was integrated into PA317 /GLUT2 and stably expressed. Conclusion A retrovirus expression vector containing GLUT-2 gene and its stable virus producing cell line PA317 /GLUT2 are successfully constructed,thus laying a foundation for the construction of "artificial islet beta cells".

syuclein is regarded as a presynaptic protein, which may play an important role in neuronal plasticity. However, abnormal accumulation of fibrillar ?-synuclein and mutation of ?-synuclein gene can affect the survival of neurons. Its aggregation and interaction with other proteins are the most critical factors. ?-synuclein is a precursor of non-amyloid component of senile plaques in Alzheimers disease. It is likely that ?-synuclein is associated with Alzheimers pathology. In this paper, we summarize the progress in the normal physical function of ?-synuclein and the mechanism in Alzheimers disease.

Objective To study the Tangniaole capsule assisted metformin on newly diagnosed type 2 diabetic patients influence blood glucose, urine glucose and insulin resistance level. Methods 80 patients with newly diagnosed type 2 diabetes mellitus were selected from January 2015 to October 2016 in our hospital. They were randomly divided into the control group and the experimental group, with 40 patients in each group. The control group was treated with metformin, and the experimental group was treated with metformin. Comparative analysis of blood and urine glucose and insulin in patients of the experimental group and the control level of resistance. Results After the corresponding treatment, the insulin resistance index in the experimental group was (2.31±0.51), and the insulin resistance index in the control group was (2.98±0.71). The insulin resistance index of the control group was significantly higher than that of the experimental group, with statistical difference (P<0.05). The fasting blood glucose level (7.31±1.02) nmol / Lin the experimental group was significantly lower than that in the control group (7.90±0.82)nmol / L, with statistical difference (P<0.05). Urine glucose levels of patients in the experimental group was significantly better than the control group, with statistical difference (P<0.05). Conclusion The application of Tangniaole capsule assisted with metformin in newly diagnosed type 2 diabetic patients in can largely reduce blood glucose and urine glucose and insulin resistance level, with further clinical promotion and application significance.