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 .

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

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

Objective To study the effect of intravenous injection of esmolol,labetalol and their combination in the treatment of ischemic heart failure (ICD).Methods 80 patients with ICD were randomly divided into treat-ment group 1 (20 cases),treatment group 2(20 cases),treatment group 3 (20 cases)and control group(20 cases). The treatment group 1 was intravenously given esmolol on the basis of routine treatment.the treatment group 2 was intravenously given labetalol on the basis of the conventional treatment.the treatment group 3 was intravenously given esmolol and labetalol on the basis of routine treatment.The control group only received routine treatment.The thera-peutic effects of the four groups were observed and compared,and the changes of blood pressure,heart rate,walking distance and ultrasonic electrocardiogram before and after treatment were recorded.Results In the treatment group 1,3 cases with marked effect,effective in 14 cases,the total effective rate was 85%.In the treatment group 2,2 cases with marked effect,effective in 14 cases,the total effective rate was 80% cases.In the treatment group 3,4 cases with marked effect,effective in 15 cases,the total effective rate was 95%.In the control group,1 case with marked effect, effective in 10 cases,the total effective rate was 55%.The treatment effect of the treatment group 1,2,3 was better than the control group (P <0.05).The treatment effect of the treatment group 1,2 was significantly lower than the treatment group 3 (P <0.05).The treatment effect between the treatment group 1 and group 2 had no significant difference (P >0.05 ).After treatment,6 min walking distance and LVEF,FS,SV and other indicators of cardiac function were significantly improved in the four groups (P <0.05 ).The four groups had no significant adverse reactions such as nausea,vomiting,liver and renal function after treatment.Conclusion Intravenous injection of esmolol,labetalol or both drugs for treatment of ICD can improve cardiac function,with no adverse reaction,and it is safe and reliable,worthy of clinical application.

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.

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.

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".

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.

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.