Maturity-onsetdiabetesoftheyoung(MODY),amonogenicformofdiabetes,hasmorethan8 well-known subtypes:MODY1( hepatocyte nuclear factor - 4A,HNF4A ),MODY2( glucokinase,GCK ),MODY3 (hepatocyte nuclear factor-1A,HNFIA),MODY4(pancreatic and duodenal homeobox,PDX),MODY5(hepatocyte nuclear factor-1B,HNFIB),MODY6(neuronal differentiation 1,NEUR0DI),MODY7(carboxyl ester lipase,CEL) and MODY8( insulin,INS). The molecular pathology,clinical manifestations and treatment of MODYs are different and reviewed.

BACKGROUND:Previous studies have shown that erythropoietin can protect neurons and promote nerve regeneration. OBJECTIVE:To explore the therapeutic effect of erythropoietin gene-modified bone marrow mesenchymal stem celltransplantation via caudal vein on rat cerebral infarction. METHODS:Western blot assay was used to identify the expression of exogenous erythropoietin in bone marrow mesenchymal stem cells. A model of middle cerebral artery occlusion was established in Wistar rats using thread method. And then, model rats were randomly divided into model group (PBS injection via the caudal vein), transplantation group (transplantation of bone marrow mesenchymal stem cellsuspension), erythropoietin group (transplantation of erythropoietin-transfected bone marrow mesenchymal stem cellsuspension). Neurologic function was assessed at 3 days, 1, 2, 3, 4 weeks after celltransplantation. Four weeks after transplantation, the rats were decapitated after anesthesia to take brain tissues for RT-PCR detection of Bcl-2/Bax gene expression. cellapoptosis was measured by TUNEL. Hematoxylin-eosin staining and fluorescence microscopy were employed to observe the survival and distribution of PKH26-labeled bone marrow mesenchymal stem cells. RESULTS AND CONCLUSION:Western blot results showed that erythropoietin-transfected bone marrow mesenchymal stem cells could express the erythropoietin in vitro. At 1, 2, 3, 4 weeks after transplantation, the neurological defect scores in the transplantation group and erythropoietin group were significantly lower than those in the model group (P<0.05, P<0.01). The expression of bcl-2 gene in the infarct region was significantly higher in the erythropoietin group than the transplantation and model groups (P<0.05), but the expression of bax was significantly decreased (P<0.05). In the erythropoietin group, the number of apoptotic cells was reduced, and the number of PKH26 positive cells was increased as compared with the other two groups (P<0.05). These findings indicate that the transplantation of erythropoietin-modified bone marrow mesenchymal stem cells via caudal vein can significantly improve the neurological function in the rats with cerebral infarction.

The pathogenesis of obesity in children is unclear.Genetic play an important role,including gene mutations,polymorphisms,epigenetics.And the other hand,environment factors such as intrauterine environment,nutrition,physical exercise,and gut microflora also affect the obesity.The genetic and environment factors have interaction,leading to the occurrence of childhood obesity and development.With the advances in molecular biology techniques and large-scale,large sample size of population screening,new obesity-related genes,single nucleotide polymorphisms,the apparent genetic markers will continue to be found,looking forward to the future predict obesity,to choose to guide effective treatment,or even contribute to the development of genetic targeting drugs.

Hemopoietic stem cells are mostly obtained from bone marrow,umbilical cord blood and peripheral blood.Physical status of final-stage liver disease patients is poor,and these patients hardly endures large wound.Thus,it is a potential method to harvest hepatocytes following transdifferentiation using peripheral blood stem cell transplantation.It is necessary to mobilize peripheral blood when enough stem cells are needed during peripheral blood stem call transplantation.Stem cell mobilization agent contains 3 types at present:chemotherapeutic drugs,polyanion preparation such as dextran sulphate,various recombinant human hematopoietic cell stimulating factors.For patients with severe liver disease,it is safe to use various recombinant human hematopoietic cell stimulating factors.Density gradient centrifugation is widely used to isolate hematopoietic stem cells.This method can obtain leukocytic cream that is rich of mononuclear cells,and primarily enrich CD34~+ cells.With the discovery of CD34~+ different antigenic determinant monoclonal antibody,immunological technique of exactly,abundantly,rapidly,positively sorting CD34~+ cells is developed quickly,such as solid phase sorting method and flow cytometry sorting.Utilizing the high reproductive activity and multi-directional differentiation,hematopoietic stem cells are amplified in vitro and induced to differentiate into abundantly hemopoietic progenitor cells in a short period,and into directional amplification of various cells such as hepatocytas for transplantation.Presently,bone marrow hematopeietic stem cells can successfully differentiate into hepatocytas.To judge whether differentiated cells were from infused stem cells in transplanted organs,the infused cells were commonly labeled before transplantation.Labeling method includes fluorescent labeling,specific staining,genetic mark and chromosome identification.Subsequently,this was identified using immunological,molecular biological,and fluorescence chemical techniques.Presently,hematopoietic stem cell transplantation in treatment of liver disease is mostly basic experiment,but has not been applied in clinic.

Objective To identify the neuroimaging determinants which could predict the occurrence of the vascular dementia (VaD). Methods The findings of cranial MRI were compared in 30 VaD patients and 30 stroke without dementia (SWD) patients by means of quantitative measurement of some indexes. The indexes of measurement included the cerebral white matter lesion (WML) area, the cerebral infarct area, the ratio of ventricle to brain(VBR) and the ratio between the areas of the corpus callosum and supratentorial brain at the midsaggittal plane. Discriminant analysis was used to search for the indexes which could contribute significantly to distinguishing the two groups. Results Small cerebral vessel disease and multi infarct were two major basal diseases of VaD in this series. The WML areas( t =-3.544, P =0.000), the left cortical infarct( Z =-2.538, P =0.011) and VBR ( t =4.475, P =0.000) were significantly higher and the corpus collsum areas( t = -4 616, P =0.000) was significantly lower in the VaD group than that in the SWD group. The indexes that could significantly discriminate the two groups was: callosal atrophy, ventricle to brain ratio, WML area, left cortical infarct area, left parietal infarct area, total cortical infarct area. Conclusions Callosal atrophy, lateral ventricle enlargement and extensive WML are important predictors of incidence of dementia in the small vessel disease; however, left cortical infarct, especially left parietal infarct, is important predictor of incidence of dementia in the multi infarct.

Objective To primarily explore the relationship between technical parameters of CT scanner and the quality of CT film.Methods Thirteen sets of CT system were tested and seven technical parameters of each CT scanner were collected,then the CT films from the tested CT system were collected and the quality of CT films were quantitatively analyzed according to "the clinical evaluative standard of CT film" by our study group.The multi-variable linear regression analysis of technical parameters of the CT scanner and the remarks of the quality of CT film was performed.Results There was linear regression relationship between the physical parameters (including CT dose index,mean CT value of water,noise,uniformity,high contrast resolution,low contrast resolution,and the linear CT value) and the score of CT film.However,among them,only low contrast resolution had statistical significant influence on the score of CT films (t= -2.460,P=0.049).Conclusion Low contrast resolution has significant influence on the quality of CT film and further study of the relationship between other physical parameters and the quality of CT film will be needed.

Objective To study the nitric oxide synthase(NOS)distribution and activity in placental villous,stem villous vessel and umbilical vessel from pregnancies with abnormal umbilical artery flow velocity waveforms and discuss the effect of NOS in the development of abnormal umbili- cal artery flow velocity waveforms.Methods Using NADPH-diaphorase histochemical method and colorimetry,we studied the distribution and activity of NOS in placental villous,stem villous vessel and umbilical vessel tissues from nine women with normal umbilical artery Doppler waveforms and eight women with abnormal umbilical artery Doppler waveforms.Results The NOS primarily dis- tributed in the syncytiotrophoblast and vessel endothelial cells.In the majority syncytiotrophoblast of normal group,the blue granules gathered to mass,appeared primarily basal in location.In the ma- jority syneytiotrophoblast of abnormal group,the blue granules diffused primarily around nucleus, the staining intension was weaker than normal.Placental villous from the abnormal group had signif- icantly lower activities of NOS than the normal group.Conclusions The lower activities and an abnormal distribution of NOS were found in the placental villous of women with abnormal umbilical artery flow velocity waveforms.The abnormity of NOS may contribute to the development abnormal umbilical artery flow velocity waveforms.

Objective:To establish a DSC method for the determination of mannityl nicotinate. Methods:Indium was used to cor-rect the instrument including temperature correction and heat flow correction. The atmosphere was nitrogen and the flow rate was 20 ml ·min-1 . The sample volume was 1-3. 5 mg with precision reached to 0. 01mg. The initial temperature was 30 ℃, raised to 120 ℃ at the rate of 10℃·min-1 , maintaining for 1 min at 120℃, and raised to 280℃ at the rate of 2. 5℃·min-1 . The results of DSC and HPLC were compared. Results:Mannityl nicotinate had a good linear relationship between the heat absorption and the amount of sam-ple within the range of 1. 05-3. 44 mg (r=0. 9990). The average recovery was 98. 86％ (RSD=1. 59％,n=9). The content of nia-cin detected by HPLC and DSC was 97. 77％ and 97. 80％, respectively, and the relative mean deviation of the two values was 0. 015％. Conclusion:The DSC method is accurate and rapid, and can be used for the determination of mannityl nicotinate like HPLC.

aused by the wrong maneuvers by inexperienced operators.

Background and purpose:Pancreatic cancer is one of the most deadly human malignant neoplasms. Resistance to chemotherapeutic drugs is a major reason responsible for poor prognosis in the treatment of pancreatic cancer patients. MicroRNA (miRNA, miR) is a family of small non-coding RNA molecules, dysregulated miRNA is associated with various tumor biological function. miR-33a has been widely reported as a metabolism-related miRNA, while its relationship with drug resistance has little understand. This study was focused on the effect of miR-33a on gemcitabine chemoresistance in pancreatic cancer to bring the novel theoretical basis to chemotherapy for pancreatic cancer.Methods:In situ hybridization and Real-time PCR were used to analyze the miR-33a expressions in pancreatic cancer tissue sample and cell lines, respectively. Cell counting kit 8 (CCK-8) assay was used to calculate the IC50 value of different pancreatic cancer cells.Results:miR-33a was down-regulated in pancreatic cancer tissue and cell lines compared with para-cancerous tissues and normal HEK293T cells. Moreover, miR-33a over expression not only could enhance the chemosensitivity to gemcitabine in pancreatic cancer cells, but also rescue the gemcitabine resistance in pancreatic cancer cells.Conclusion:Down regulation of miR-33a in pancreatic cancer decreases the chemosensitivity to gemcitabine, resulting in development of acquired gemcitabine chemoresistance. It provides the theoretical basis to develop a new molecular targeted drug to combine with chemotherapy for pancreatic cancer.