To investigate the effect of high concentration glucose and AGEs on VCAM 1 expression of mouse cardiac microvascular endothelial cells, the VCAM 1 expression was measured by using FACS after the endothelial cells were incubated with high concentration glucose, AGEs alone or combined respectively, the TNF ? level of cultured medium was measured by using RIA. The results showed that the VCAM 1 expression and TNF ? level increased significantly in high concentration glucose and AGEs group and increased more over in combined group. It is suggested that High concentration glucose and AGEs could induce the VCAM 1 expression, and has synergistic action. The increased level of TNF ? coincident with the increased VCAM 1 expression,is probably one of the factors in the mechanism of increased VCAM 1 expression.

Objective To explore the characteristics and laws of burned children,and to find the coping strategies on preventing and reducing the incidence of pediatric burns.Methods The age distribution,burn causes, degree of burn,easy -happening months and on -site treatment of 978 children under 12 years old were retrospective-ly analyzed.Results 978 pediatric burns patients made up 55.4% of all burned patients among which there were 601 male patients and 377 female patients.Most of them were 1 -3 years old.The main reason of burn was hot liquid. Most of them were minor and moderate burned.The easy -happing months were from May to September.Only 11.0%of these children were correctly treated.Most burn children were from countryside.Conclusion The main reasons of children being burned are poor management of hot liquid,carelessness and the indifference of safety consciousness. The cases of burned children are regular.It is important to strengthen the publicity and education of precaution and the consciousness of safety protection of custodian.The publicity of knowledge about burning safety and correct on -site treatment are important for prevention and cure of burned children.

Objective:To study the change of plasma cyclic nucleotide in patients of rheumatic heart disease with pulmonary hypertension and to evaluate the relationship between plasma cyclic nucleotides and hemodynamics. Methods:Plasma cGMP and cAMP were examined by radioimmunoassay, hemodynamic data were obtained with Swan Ganz catheter and the HP compact configurable monitors. Results:Plasma cyclic nucleotide increased in patients of rheumatic heart disease with pulmonary hypertension and had an obvious correlation with the level of PAPM, PVR, PAWP and CI. Conclusion:Increased cAMP and cGMP is one of the pathophysiological characters of pulmonary hypertension, cAMP and cGMP, as the second messenger, may play an important regulating role in the development of pulmonary hypertension.

Objective To investigate the clinical characteristics of methylmalonic academia in adolescence cases. Methods 4 cases were diagnosed methylmalonic academia by gas chromatography- masss pectrogram whose clinical, manifestations and treatment process were analyzed. Results The main clinical manifestations in 4 cases with methylmalonic academia were intellect impairment,epilepsy, pyramid signs; 2 of them suffered with hypopsia and optic atrophy, one of them suffered with papilledema. Symptoms were improved after treated with cobamamide and L-carnitine in all the 4 cases 1 months later. Conclusions The main clinical characteristics of methylmalonic academia in adolescence were intellect impairment, epilepsy and pyramid signs. The symptoms could be improved after treatment.

Down syndrome cell adhesion molecule (DSCAM) acts as a netrin-1 receptor and mediates attractive response of axons to netrin-1 in neural development. However, the signaling mechanisms of netrin-DSCAM remain unclear. Here we report that AMP-activated protein kinase (AMPK) interacts with DSCAM through its γ subunit, but does not interact with DCC (deleted in colorectal cancer), another major receptor for netrin-1. Netrin-treatment of cultured cortical neurons leads to increased phosphorylation of AMPK. Both AMPK mutant with dominant-negative effect and AMPK inhibitor can significantly suppress netrin-1 induced neurite outgrowth. Together, these findings demonstrate that AMPK interacts with DSCAM and plays an important role in netrin-1 induced neurite outgrowth. Our study uncovers a previously unknown component, AMPK, in netrin-DSCAM signaling pathway.
AMP-Activated Protein Kinases ; antagonists & inhibitors ; genetics ; metabolism ; Animals ; Cell Adhesion Molecules ; genetics ; metabolism ; Cells, Cultured ; HEK293 Cells ; Humans ; Mice ; Nerve Growth Factors ; pharmacology ; Netrin-1 ; Neurites ; physiology ; Neurons ; cytology ; drug effects ; metabolism ; Phosphorylation ; Protein Binding ; Protein Kinase Inhibitors ; pharmacology ; RNA Interference ; RNA, Small Interfering ; Recombinant Fusion Proteins ; biosynthesis ; genetics ; Signal Transduction ; drug effects ; Transfection ; Tumor Suppressor Proteins ; pharmacology

The nervous system is one of the most complicated organ systems in invertebrates and vertebrates. Down syndrome cell adhesion molecule (DSCAM) of the immunoglobulin (Ig) superfamily is expressed widely in the nervous system during embryonic development. Previous studies in Drosophila suggest that Dscam plays important roles in neural development including axon branching, dendritic tiling and cell spacing. However, the function of the mammalian DSCAM gene in the formation of the nervous system remains unclear. Here, we show that Dscam ( del17 ) mutant mice exhibit severe hydrocephalus, decreased motor function and impaired motor learning ability. Our data indicate that the mammalian DSCAM gene is critical for the formation of the central nervous system.
Animals ; Cell Adhesion Molecules ; genetics ; metabolism ; Corpus Callosum ; metabolism ; pathology ; Genotype ; Hydrocephalus ; genetics ; metabolism ; pathology ; Mice ; Mice, Knockout ; Motor Activity ; genetics ; physiology ; Mutation

Mutations in the Fused in sarcoma/Translated in liposarcoma gene (FUS/TLS, FUS) have been identified among patients with amyotrophic lateral sclerosis (ALS). FUS protein aggregation is a major pathological hallmark of FUS proteinopathy, a group of neurodegenerative diseases characterized by FUS-immunoreactive inclusion bodies. We prepared transgenic Drosophila expressing either the wild type (Wt) or ALS-mutant human FUS protein (hFUS) using the UAS-Gal4 system. When expressing Wt, R524S or P525L mutant FUS in photoreceptors, mushroom bodies (MBs) or motor neurons (MNs), transgenic flies show age-dependent progressive neural damages, including axonal loss in MB neurons, morphological changes and functional impairment in MNs. The transgenic flies expressing the hFUS gene recapitulate key features of FUS proteinopathy, representing the first stable animal model for this group of devastating diseases.
Aged ; Aging ; genetics ; metabolism ; pathology ; Amyotrophic Lateral Sclerosis ; genetics ; metabolism ; pathology ; Animals ; Animals, Genetically Modified ; Disease Models, Animal ; Drosophila melanogaster ; genetics ; metabolism ; Gene Expression ; Humans ; Microscopy, Electron, Scanning ; Motor Neurons ; metabolism ; pathology ; Mushroom Bodies ; metabolism ; pathology ; Mutant Proteins ; genetics ; metabolism ; Mutation ; Photoreceptor Cells, Invertebrate ; metabolism ; pathology ; Plasmids ; RNA-Binding Protein FUS ; genetics ; metabolism ; Recombinant Fusion Proteins ; genetics ; metabolism ; Retinal Degeneration ; pathology ; physiopathology ; Transfection

Group A Streptococcus (GAS) is a very important pathogen, especially for children.On a global scale, GAS is an important cause of morbidity and mortality.But the burden of disease caused by GAS is still unknown in China and also has not obtained enough attention.For this purpose, the expert consensus is comprehensively described in diagnosis, treatment and prevention of GAS diseases in children, covering related aspects of pneumology, infectiology, immunology, microbiology, cardiology, nephrology, critical care medicine and preventive medicine.Accordingly, the consensus document was intended to improve management strategies of GAS disease in Chinese children.