Neurofibromatosis type 1 is a term of Von Recklinghausenan.It is an autosomal dominant inherited disease which derived by neural crest cell.Prevalence of this disease is 1/3000 1/3500 and is a disease with the highest mutation rate.The pathogenesis of neurofibromatosis type 1 is associated with the deficiency of NF1 gene.Recently,the genetics and genomics research of neurofibromatosis make a great progress.With the development of gene linkage and position cloning technology,the gene sequence of neurofibromatosis type 1 has been found.Recent research of genetics and genomics of NF1 and the structure and function,abnormal expression,the relation of genotype and phenotype,the mutation sensitivity domain of NF1 gene were reviewed.

[Summary] Glucagon-like peptide-1 ( GLP-1 ) agents play important roles in glycemic control in type 2 diabetes. Moreover, these agents also show various protective effects on cardiovascular system. GLP-1 agents improve vascular endothelial function, ameliorate the risk factors of cardiovascular disease including obesity, hyperglycemia, dyslipidemia and hypertension, delay the occurrence and progression of atherosclerosis, and protect against cardiac ischemia-reperfusion injury and heart failure. Therefore, GLP-1 agents may have beneficial effects on cardiovascular diseases at different stages and in multiple aspects.

To study the chemical constituents of Cirsium setosum (Willd.) MB., 70% ethanol extract of the aerial parts was subjected to column chromatography. One new phenylpropanoid glycoside, sinapyl alcohol 9-O-(E)-p-coumaroyl-4-O-beta-D-glucopyanoside (1) was isolated, along with three known compounds: lycoperodine-1 (2), apigenin-7-O-(6"-(E)-p-coumaroyl)-beta-D-galactopyranoside (3) and quercetin (4). The structures were elucidated on the basis of spectral and chemical evidence. Compound 2 was obtained from Cirsium genus for the first time, compounds 3 and 4 were obtained from this plant for the first time.

Objective To investigate the relationship between exposure intensity and illumination time of blue light and replicative senescence of rat retinal pigment epithelial (RPE) ceils.Methods Thirty-six 12-14 weeks Wistar rats were kept in the cage with a blue-light bulb [(450±10) nm],and were randomly divided into four groups (no light,nature light,500 lx light and 1000 lx light illumination),each has nine rats.The rats in each group were further divided into three subgroups according to illumination time (one month,two months or three months).Eyeballs were collected after intraperitoneal injection of 10% chloral hydrate.The right eye of each rat was embedded in paraffin and sectioned for hematoxylin-eosin (HE)staining,while frozen sections of the left eye were stained for the senescence-associated β-galactosidase (SA-β-Gal).The data were analyzed by SPSS11.5 statistical software.Results The amounts of SA-β-Gal positive RPE cells were significantly different between all groups under the same illumination time 17 (P=0.000),and between all subgroups of different illumination time with same exposure intensity (P＜0.01)except for the control group (no light).Conclusion Blue-light can induce replicative senescence in rat RPE cells in an intensity and time-dependent manner.

Animals ; Calpain ; Humans ; Nervous System Diseases ; chemically induced ; Organophosphate Poisoning

Objective: To observe the clinical efficacy of Jin's three-needle therapy on post-stroke cognitive impairment (PSCI) and the effect on neuroelectrophysiology (event-related potentials). Methods: A total of 60 PSCI patients were selected and divided into a treatment group and a control group according to the method of random number table, with 30 cases in each group. The patients in the control group received routine treatment while the patients in the treatment group received additional Jin's three-needle therapy. The treatment for both groups lasted four weeks. Mini-mental state examination (MMSE) and Montreal cognitive assessment (MoCA) scores as well as amplitude and latency of potential 300 (P300) were adopted to compare the between-group results before and after treatment. Results: Before treatment, there were no significant differences (all P>0.05) in MMSE and MoCA scores, P300 latency and P300 amplitude between the two groups. After 4 weeks of treatment, the MMSE and MoCA scores and P300 amplitudes were improved in both groups, and the P300 latencies became shorter. The results showed significant intra-group and between-group differences (all P<0.05). Conclusion: Based on the routine treatment, Jin's three-needle therapy is effective for PSCI. The mechanism is probably through its regulation on the patients' neuroelectrophysiology.

This study aimed to investigate the effects of fusion proteins GnRH-GRP(G3G6)and HSP65-STEAP1(HST1)on dendritic cells(DC)and the sensitization of DCs to B16F10 melanoma. The fusion proteins G3G6 and HST1 were obtained using the previous engineering strains in our laboratory. Group by unsensitized DC(US-DC), the G3G6 fusion protein sensitized DC, the HST1 fusion protein sensitized DC(HST1-DC)and the combined sensitized DC(GH-DC), the mouse bone marrow-derived DCs were sensitized with fusion protein to obtain the fusion protein sensitized DC vaccines. B16F10 melanoma cells were transplanted into C57BL/6J male mice to construct a melanoma model(1×106 cells per mouse), and DC vaccine was injected for treatment. The antitumor efficacy of DC vaccine was explored by in vitro and in vivo experiments. Flow cytometry analysis showed that the fusion protein can effectively stimulate DC into differentiation and maturation; in the animal experiment, the inhibition rate of melanoma treated with G3G6-DC was 35. 75%, that of HST1-DC group and combination group were 34. 03% and 55. 74%. It was initially proved that both G3G6-DC and HST1-DC can effectively inhibit the growth of transplanted tumors of melanoma B16F10 cells in mice, and the combination therapy is superior to the single therapy.

Objective: To study the pharmacokinetics and tissue distribution of procesed Strychni Semen in rats after multiple administration at clinical dose. Methods: The rats were given procesed Strychni Semen by intragastric administration for single and several times. The plasma concentrations of brucine and strychnine in plasma and tissues of rats were determined by UPLC-Q-Orbitrap HRMS method, and the organizational distribution differences were compared to explore whether there was accumulation in the body. Results: After single intragastric administration of procesed Strychni Semen, the main pharmacokinetic parameters of brucine and strychnine were as follow: t1/2 was 10.59 and 8.39 h, tmax was 0.77 and 0.64 h, t1/2Ka was 5.38 and 2.63 h, Cmax was 2.97 and 10.83 ng/L, AUC0-t was 87.36 and 172.24 ng∙h/L, CL/F was 40 637.08 and 38 370.26 L/(h∙kg); Brucine and strychnine in processed Strychni Semen reached a steady state after 3 d of administration in rats. After the last administration, the main pharmacokinetic parameters of brucine and strychnine in rats were as follow: t1/2 was 7.07 and 4.75 h, tmax was 0.48 and 0.46 h, t1/2Ka was 3.23 and 1.09 h, Cmax is 5.77 and 34.83 ng/L, AUC0-t was 32.80 and 107.86 ng∙h/L, and CL/F was 75 920.52 and 43 871.54 L/(h∙kg). Compared with the single administration, the content of brucine and strychnine in the liver and kidney tissues was significantly reduced after multiple administrations, and there was no significant change in other tissues. Conclusion: The pharmacokinetics of brucine and strychnine in healthy rats is consistent with one-compartment model. Both of them have the pharmacokinetic characteristics of fast absorption in rats. After 3 d of administration, the serum concentrations of brucine and strychnine reached steady state, and the blood concentration was increased continuously with the increase of administration times. The content of each tissue did not increase after single and multiple administrations. It is suggested that long-term administration of brucine and strychnine will not cause the superposition of the concentration of brucine and strychnine in the blood of rats, which may lead to the occurrence of poisoning.

Background Protein kinase B (Akt) is the center of multiple cellular signaling pathways,and it participates in the regulation of cell function,such as proliferation,migration,and metabolism of cells.Phosphoinositide 3-kinase (PI3K) promotes activation of Akt and therefore triggers many signal pathways.PI3K inhibitor can silent Akt,but whether it can affect the biological behavior of lens epithelial cells (LECs) during the posterior capsular opacity (PCO) is worthy of investigation.Objective This study was to explore the effect of LY294002,a PI3K inhibitor,on Akt activation in human LECs.Methods Human LECs strain,HLEC-B3 cells,were cultured and passaged.The cells were incubated to 96-well plate for 24 hours,then LY294002 was added with the final concentration at 0,10,20,30,40,50,60,70,80 μmol/L,respectively.After incubation for 24 hours,cell counting kit-8 (CCK-8) was used to detect the inhibitory rate of cell proliferation.Transforming growth factor-β2 (TGF-β2) of 10 μg/L was added in the medium of cells as TGF-β2 group,TGF-β2 + LY294002 (20 μmol/L) was used to coculture the other group of cells,and the cells without TGF-β2 and LY294002 served as the control group.Phosphorylation of Akt (p-Akt) in the cells was detected by laser scan confocal immunofluorescence microscope.The expression level of p-Akt was evaluated using Western blot.Results CCK-8 assay showed that the A value of the cells was gradually reduced with the increase of LY294002 concentration (Fgroup =9.72,P =0.00),but the A value was significant raised along with the lapse of time (Ftime =1737.54,P=0.00).Confocal immunofluorescence revealed that a little of p-Akt was expressed in the control cells.After induced by TGF-β2,lots of red fluorescence of p-Akt was seen in cells around the cell membranes.But in the TGF-β2+LY294002 co-culture cells,the fluorescence of Akt was much weaker.Western blot showed that the expression level of p-Akt was 0.91±0.08,1.48±0.13 and 0.95±0.19 in the control group,TGF-β2 group and TGF-β2 +LY294002 group,respectively,with a significant difference among the three groups (F =15.04,P =0.00).Conclusions LY294002 can inhibit the activation of Akt induced by TGF-β2.LY294002 may have utility in the prevention and treatment of PCO.

Objective To introduce the technique and treatment outcome of the fingertip soft tissue defect with improved transverse palmar island flap.Methods From January 2011 to February 2013,15 cases(17 fingers) fingertip defect were repaired by improved transverse palmar island flap,the vascular pedicled of flap was a side of the neurovascular bundle,the flap revolved 90° to coversoft tissue defect.Defect area:1.2 cm × 0.8 cm to 1.7 cm × 1.6 cm.The time between the injury and admission was 40 minutes to 27 hours.Five cases were repaired defection with flap immediately,10 cases were repaired defection in stage Ⅱ.Results The wounds of injury in 15 cases get primary healing,and all the flaps were successfully survived.The 13 cases were followed up for 6 months to 14 months.The blood circulation of flap was fine,and the fingertip shape as well.The two point discrimination of the skin pulp was 6 mm to 11 mm.Conclusion The improved transverse palmar island flap for repairing transverse finger pulp defect and a small area of skin defect in patients.The procedure has a simple surgery,donor area don't skin graft,postoperative appearance and hand finger satisfactory functional recovery,etc.