Glycogen storage diseases (GSD) are a group of inherited disorders characterized by enzyme defects that affect the glycogen metabolism. Hypoglycemia, hepatomegaly, splenomegaly and growth retardation are the main clinical manifestations. The enzymes affecting glycogen synthesis and degradation are varied.Consequently, the clinical manifestations are different because of different enzyme defection. Most patients treated regularly can obtain the normal growth and grow to adults, but still can be accompanied long-term complications by adulthood. Now some scientists find new modified cornstarch is superior to standard therapy in maintaining blood glucose levels. Gene therapy is still in the experimental animals.

Triple negative breast cancer(TNBC) is defined by the lack of estrogen receptor (ER) , progesterone receptor (PR) and human epidermal growth factor receptor-2 ( Her-2) expression. It is a subtype of tumor with distinct clinical and pathologic features and lacks effective targeted therapies. It is non - sensitive to endocrine therapy and targeted therapy drug herceptin, leaving chemotherapy as the mainstay of treatment The feature and the treatment of TNBC is the hotspot of resent research.

C-termini of proteins often play an important role in various biological processes, such as the transcription and translation from DNA to protein and also participating in various biological regulations. The determination of protein C-terminus is so crucial because it provides not only distinct functional annotation, but also a way to monitor the proteolysis-modified proteins. Based on the biological mass spectrometry, a series of novel methods and technologies were developed both for qualitative and quantitative analyses of protein C-terminus. These methods or technologies can be applied to accurate and effective protein C-terminus profiling, including the sequences and quantitative information of C-termini, which reveals the biological function of C-termini in life's activities and provides a better understanding of the degradation of mature proteins. Combined with our research, this review highlights the improvements in C-terminal proteomics study in the past decades, including the methodologies for recognition and identification of C-terminus, as well as the enrichment strategies for protein C-terminus.
Mass Spectrometry ; Protein Processing, Post-Translational ; Proteins ; chemistry ; Proteolysis ; Proteomics

OBJECTIVE:To observe the effects of Xingnaojing injection on the efficacy and safety of patients with acute cere-bral infarction merge blood stasis syndrome. METHODS:70 patients with acute cerebral infarction merge blood stasis syndrome were randomly divided into observation group(35 cases)and control group(35 cases). Control group received conventional treat-ment according to 2014 edition of Chinese guideline for diagnosis and management of acute ischemic stooke. Patients within throm-bolysis time window were given intravenaus infusion of rt-PA,and those out of thrombolysis time window orally received 0.3 g As-pirin tablet every evening,then 0.1 g after 1 week+40 mg Atorvastatin calcium tablet every evening,and if patients have arfficulty suallowing,gavage administiation was implemented. Observation group additionally received 30 ml Xingnaojing injection,adding in-to 250 ml 0.9% Sodium chloride injection,intravenous infusion,once a day. The treatment course for both groups was 14 d. Clini-cal efficacy,γ-interferon(IFN-γ),interleukin-13(IL-13),interleukin-4(IL-4),high-sensitivity C-reactive protein(hs-CRP)lev-els,the US National Institutes of Health Stroke Scale(NIHSS)rating,improved Barthel index(BI),infarction size before and af-ter treatment and the incidence of adverse reactions in 2 groups were observed. RESULTS:There was no significant difference in the total effective rate and incidence of adverse reactions in 2 groups(P>0.05). Before treatment,there were no significant differ-ences in IFN-γ,IL-13,IL-4,hs-CRP levels,the NIHSS rating,BI and infarction size in 2 groups(P>0.05). After treatment,the IFN-γ,hs-CRP levels,the NIHSS rating and infarction size in 2 groups were significantly lower than before,and observation group was lower than control group,IL-13,IL-4 and BI were significantly higher than before,and observation group was higher than control group,the differences were statistically significant (P<0.05 or P<0.01). CONCLUSIONS:Based on conventional treatment,Xingnaojing injection shows obvious efficacy the efficacy and safety of patients with acute cerebral infarction merge blood stasis syndrome,it can reduce the levels of inflammatory cytokines,improve neurological deficits,daily living skills and in-farct volume,with good safety.

Safety assessment in clinical trials is dependent on an in-depth analysis of the adverse events to a great extent. However, there are difficulties in summary classification, data management and statistical analysis of the adverse events because of the different expressions on the same adverse events caused by regional, linguistic, ethnic, cultural and other differences. In order to ensure the normative expressions, it's necessary to standardize the terms in recording the adverse events. MedDRA (medical dictionary for regulatory activities) has been widely recommended and applied in the world as a powerful support for the adverse events reporting in clinical trials. In this paper, the development history, applicable scope, hierarchy structure, encoding term selection and standardized query strategies of the MedDRA is introduced. Furthermore, the practical process of adverse events encoding with MedDRA is proposed. Finally, the framework of statistical analysis about adverse events is discussed.

Objective To understand the endemic situation of schistosomiasis after its transmission interruption in Baoying County,so as to provide the evidence for adjusting the prevention and control strategy. Methods The data of schistosomiasis surveillance in Baoying County from 1988 to 2015 were collected and retrospectively analyzed. Results The schistosomiasis surveillance has been established since it was interrupted in Baoying County in 1987. The total surveyed area with Oncomelania hupensis snails was 21 269.95 hm2,but no living snails were found. The number of schistosomiasis examinations of residents was 163 196 person?times,and 270 person?times were positive in serum test,26 person?times were positive in stool test. No positive cases were found since 1997. The number of schistosomiasis tests of livestock was 2 180 cattle?times but no infection was found. Conclusion No schistosome infected snails or livestock are founded in Baoying County for last 28 years,and no patients are founded for last 11 years. The effect of schistosomiasis control is steady.

Objective To analyze academic influence of SCI papers of TCM colleges and universities in the recent 10 years;To provide references for scientific research and academic exchange. Methods According to the evaluation ranking of degree and graduate education development center of Ministry of Education published in 2012 and standardization of InCites database, 10 Chinese medicine colleges and universities were selected. InCites platform was used to select 13 representative indicators and to analyze the cited cases, H index and the cooperation of the SCI papers published in 2006-2015. Results The SCI papers published by 10 TCM colleges and universities have been growing rapidly since 2010, and the average influence of standardized citations was 0.76. The average percentage of cited papers was 67%, which was slightly lower than that of 71%in clinical medicine papers of mainland of China. H index was 30. The average percentage of international cooperation papers in TCM colleges and universities was 17.71%, which was lower than the percentage of international cooperation papers in clinical medicine of mainland of China (22.85%). Conclusion In recent 10 years, the academic level of TCM colleges and universities in our country has been developing rapidly. However, there is still a need to further explore international perspectives, focus on research frontiers, strengthen international cooperation, carry out innovative research and improve academic influence.

OBJECTIVE:To study the effects of Anzi mixture on Toll like receptor 4(TLR4)/myeloid differentiation factor 88 (MyD88)/nuclear facter-κB(NF-κB)signaling pathway of antiphospholipid antibodies(APA)positive abortive mice,and to inves-tigate the mechanism of anti-APA positive abortion. METHODS:BALB/c mice(female)were randomly divided into blank control group,model group,aspirin group (positive control,0.0195 g/kg) and Anzi mixture low-dose,medium-dose and high-dose groups (37.7,75.4,150.8 g/kg,calculated by crude drug),with 10 mice in each group. Except for blank control group,other groups were given human β2-glycoprotein Ⅰ as derivant to establish APA positive abortion model. From the first day of pregnancy, treatment groups were given relevant medicine intragastrically,and blank control group and model group were given constant vol-ume of normal saline intragastrically,once a day,for consecutive 9 d. mRNA and protein levels of TLR4,myeloid differentiation 2 (MD2),MyD88 and NF-κB in placental tissue of mice were determined by RT-PCR and immunohistochemical method. RE-SULTS:Compared with blank control group,mRNA and protein expression of TLR4,MD2,MyD88 and NF-κB in placental tis-sue were increased markedly in the model group(P<0.01). Compared with model group,mRNA and protein expression of TLR4, MD2 and MyD88 in aspirin group and Anzi mixture low-dose and medium-dose groups were decreased significantly as well as the protein expression of TLR4 in Anzi mixture high-dose group and the protein expression of NF-κB in all medicine groups(P<0.05 or P<0.01). mRNA expression of TLR4 and MD2 and the protein expression of MD2 and MyD88 in Anzi mixture low-dose groups were lower than those in aspirin group (P<0.05 or P<0.01). CONCLUSIONS:Anzi mixture can inhibit TLR4/MyD88/NF-κB signaling pathway of APA positive abortive mice,which may be one of anti-APA positive abortion mechanisms.

Recent studies have shown that pituitary hormones, including thyroid stimulating hormone (TSH), follicle stimulating hormone(FSH), adrenocorticotropic hormone(ACTH), and oxytocin(OT)may actually bypass their target endocrine organs and affect the skeleton directly. Therefore, a new conception, pituitary-bone axis is proposed. This breakthrough sheds a new light on the function of pituitary hormones and the pathogenesis of osteoporosis associated with hyperthyroidism, menopause or pregnancy, and even osteonecrosis after using glucocorticoids. In addition, it is conducive to give the reference guidance for clinical treatment of metabolic bone diseases and new drug development.

BACKGROUND:Bone marrow mesenchymal stem cels have good proliferation and paracrine functions, which have irreplaceable advantages in the treatment of intestinal diseases. OBJECTIVE:To explore the effects of bone marrow mesenchymal stem cel transplantation on intestinal ischemia-reperfusion injury in rats. METHODS:Forty-eight Sprague-Dawley rats were enroled to make animal models of ischemic reperfusion injury of the intestine, and then model rats were randomized into experimental and control groups. After modeling, 1 mL bone marrow mesenchymal stem cels or the same volume of normal saline were injected into the intestinal mucosa of rats in the two groups, respectively. At hours 0, 2, 6, 24, 72, 120 after injection, serum diamine oxidase,tumor necrosis factor α, and D-lactic acid levels were detected by ELISA method. At 24 hours after injection, rat intestinal tissues were taken and observed pathologicaly under light microscopy, and their close connections were observed under transmission electron microscope. ZO-1 protein levels were detected by immunohistochemistry method. RESULTS AND CONCLUSION:Compared with the control group, the serum diamine oxidase, tumor necrosis factor α, and D-lactic acid levels were significantly lower in the experimental group at hours 6 and 24 after injection (P < 0.05). Intestinal necrosis, vilous edema, intestinal congestion and inflammatory cel infiltration in the experimental group were milder than those in the control group. In addition, the ZO-1 protein expression in the experimental group was higher than that in the control group. Experimental results show that bone marrow mesenchymal stem cel transplantation into the intestinal mucosa can improve the intestinal mucosal permeability in rats with intestinal ischemia-reperfusion injury.