There are twenty-one gene-associated sites of hereditary spastic paraplegia (HSP) that have been reported, including eleve gene-associated sites of autosomal dominant inheritance, seven gene-associated sites of autosomal recessive and three gene-associated sites of X2 linked recessive. Nine genes of HSP have been cloned as followed, atlastin, spastin, paraplegin, L1CAM, PLP, Hsp60, maspardin, TRAK1 and so on.

Air Pollutants ; analysis ; Animals ; Child ; Esters ; toxicity ; Humans ; Liver ; drug effects ; Phthalic Acids ; analysis ; metabolism ; toxicity ; Reproduction ; drug effects ; Soil Pollutants ; analysis ; Thyroid Gland ; drug effects ; Water Pollutants, Chemical ; analysis

Objective To test the reliability and validity of the Chinese Version of the Fatigue severity scale (FSS)in patients with cerebral infarction.Methods The FSS was translated into Chinese language and the reverse translation was done by several experts.Validity,dimensionality,and reliability tests were implemented in 153 cases of cerebral infarction.Results One component was extracted in factor analysis,and the total cumulative contribu- tion was 64.982%.Based on the Mokken Scale analysis for Polytomous items analyses,the scale was found to be u- nidimensional and scale H is 0.6125,Cronbach?of the scale is 0.9287.Conclusion The psychometric proper- ties(reliabilities and validities)of FSS Chinese Version was satisfactory and seemed to be adaptable to Chinese cere- bral infarction patients.

Objective To investigate the effects of FKBP12.6 gene transfection on the contractility of ventricular myocytes of rats with heart failure.Methods Rat model of heart failure was established by a surgical operation of abdominal aortic stenosis.The enzymatic hydrolysis was employed to isolate the rats' ventricular myocytes.Mediated by adenovirus the FKBP12.6 gene was transfected into ventricular myocytes.Ad-GFP infected cardiac myocytes from heart failure rats and normal rats were used as control.Western blotting and reverse transcriptase-polymerase chain reaction(RT-PCR) were used to detect the specific overexpression of FKBP12.6.Transfected ventricular myocytes were loaded with the membrane-permeant Ca2+ dye X-rhod-1-AM.Under the condition of field stimulation,the laser confocal microscope in line-scan and plane-scan mode was used to detect the Ca2+ transients and myocytes contraction.Results The mRNA and protein levels of FKBP12.6 in the myocytes of rats with heart failure were significantly lower than that in normal myocytes(0.47?0.08 vs 0.96?0.12,0.25?0.04 vs 0.48?0.07,P

AIM:To study the effects of FK506 binding protein 12.6(FKBP12.6) overexpression on the performance of sarcoplasmic reticulum(SR) in ventricular myocytes of rats with heart failure.METHODS:The adenovirus(Ad)-mediated gene transfer was used to overexpress FKBP12.6 in ventricular myocytes of rats with heart failure.Western blotting and reverse transcriptase-polymerase chain reaction(RT-PCR) analysis were used to reveale specific overexpression of FKBP12.6.X-rhod-1-AM was used as the Ca2+ indicator,and cells were viewed under a confocal microscope.RESULTS:Adenovirus mediated overexpression of FKBP12.6 resulted in a 5-fold increase in relative FKBP12.6 mRNA levels and a 4-fold increase in relative FKBP12.6 protein levels at 48 h after transfection compared with control.The amplitude of the fluorescence [Ca2+]i transient was significantly increased in Ad-FKBP12.6-GFP cardiomyocytes compared with Ad-GFP myocytes(peak F/F0,3.16?0.42 vs 1.43?0.38,P

OBJECTIVETo investigate the effect of Jiangtang Yishen Recipe (JTYSR) on high insulin induced cell proliferation of human glomerular mesangial cells (HMCs) and the expression of insulin receptor substrate 1 (IRS-1) and phosphatidylinositol-3-kinase (PI-3K).

METHODSHMCs were divided into 4 groups, i.e., the negative control group, the high insulin model group, the JTYSR group, and the LY294002 group. The concentration of insulin, JTYSR, and LY294002 was respectively confirmed by pre-experiment. Different culture solution was respectively added for different groups. RPMI1640 culture solution was added for HMCs in the negative control group, while HMCs in the rest 3 groups were cultured by 100 nmol/L insulin for 24 h. Meanwhile, HMCs from the JTYSR group and the LY294002 group were exposed to 125 mg/L JTYSR and 80 micromol/L LY294002 respectively for further 48 h. The proliferation of HMCs was detected by MTT and flow cytometry. The protein expression of IRS-1 and PI-3K in HMC was detected by immunohistochemical assay and Western blot. Results The proliferation of HMCs induced by high insulin could be significantly lowered, and the protein expression of IRS-1 and PI-3K could be down-regulated in the JTYSR group and the LY294002 group (P <0.01). Compared with the LY294002 group, the protein expression of IRS-1 and PI-3K could be slightly down-regulated in the JTYSR group (P <0.05).

CONCLUSIONJTYSR could lower high insulin induced proliferation of HMCs, and its mechanism might be related to insulin signaling pathway.

Cell Proliferation ; drug effects ; Chromones ; Drugs, Chinese Herbal ; pharmacology ; Humans ; Insulin Receptor Substrate Proteins ; metabolism ; Mesangial Cells ; physiology ; Morpholines ; Phosphatidylinositol 3-Kinase ; metabolism ; Phosphatidylinositol 3-Kinases ; metabolism ; Signal Transduction

This article aimed to study the antigenicity of nucleocapsid proteins (NPs) in six pathogenic phleboviruses and to provide theoretical evidence for the development of serological diagnostic reagents. NPs of six pathogenic phleboviruses were expressed and purified using a prokaryotic expression system and rabbits were immunized with individual recombinant NPs. Cross-reactions among NPs and rabbit sera were determined by both indirect ELISA and Western blotting analyses, and the sera titer was determined by indirect ELISA. Furthermore, sera from SFTS patients were also detected by each recombinant NP as a coating antigen using indirect ELISA. The cross-reactions and the sera titer were subsequently determined. Both the concentration and purity of recombinant NPs of six pathogenic phleboviruses met the standards for immunization and detection. The results of indirect ELISA and Western blotting showed that each anti-phlebovirus NP rabbit immune serum had potential serological cross-reactivity with the other five virus NP antigens. Furthermore, the sera from SFTS patients also had cross-reactivity with the other five NP antigens to a certain extent. Our preliminary study evaluated the antigenicity and immune reactivity of six pathogenic phleboviruses NPs and laid the foundation for the development of diagnostic reagents.
Animals ; Antibodies, Viral ; immunology ; Antigens, Viral ; genetics ; immunology ; Cross Reactions ; Humans ; Nucleocapsid Proteins ; genetics ; immunology ; Phlebotomus Fever ; diagnosis ; immunology ; virology ; Phlebovirus ; classification ; genetics ; immunology ; isolation & purification ; Rabbits

Objective To identify the imbalance of T cell-specific transcription factors T-bet/GATA-3,and to explore the modulation with dexamethasone and imiquimod in CD4+T cells from ovalbumin (OVA)sensitized mice.Methods CD4+T cells were obtained fromsingled-cell suspension of spleen(after lysis of RBC).ELISA assay was used to detect the concentrations of IL-4,IL-5 and IFN-γin superna tants and cell pellets,and the expression of T-bet and GATA-3 was detected by Western blot.Resuits In the control group,tIle low levels of IFN-γ were detected in the supernatants during 24 h.In OVA treatment group,the concentrations of IL-4,IL-5 were increased significantly,and the concentrations of IFN-γ were always low in the supernatants.In the dexamethasone treatment group,the concentrations of IFN-γ,IL-4 and IL-5 were all low in the supernatants during 24 h.In the imiquimod treatment group,the concentrations of IFN-γ were increased significantly,and the concentrations of IL-4 and IL-5 were decreased in the super natants.It worked at 6 h,and achieved the peak at 12 h,lasted over 24 h.In the control group,the expres sions of T-bet and GATA-3 were detected in CD4+T cells during 24 h.In OVA treatment group,the expressions of T-bet were decreased,and that of GATA-3 were increased rapidly in CD4+T cells.In dexam ethasone treatment group,the expressions of T-bet were always low in CD4+T cells,and that ofGATA-3 were no change during 24 h.In imiquimod treatment group,the expressions of T-bet were increased,andthat of GATA-3 were decreased in CD4+T cells.The protein expressions worked at 6 h.and achieved the peak at 12 h,lasted over 24 h.Conclusion The imbalance T cell-specific transcription factors T-bet/GA-TA-3 contributes to both high expression of GATA-3 and low expression of T-bet in CD4+T cells from OVA sensitized mice.Dexamethasone treatment inhibits the expression of T-bet in CD4+T cells and has no func tion in GATA-3.Imiquimod treatment modulates key master switches GATA-3 and T-bet that results in com mitting T helper cell to a TH 1 phenotype and imiquimod may play a key role in the regulation of TH2 cytokine responses in asthma.

Cervical spondylotic myelopathy (CSM) is a common cause of spinal cord dysfunction clinical disease. Surgery is the main therapeutic tool for CSM. However, there are obvious differences in clinical functional recovery after operation. For the past few years, the influence factors of prognosis in cervical spondylosis myelopathic has been widely concerned. Age, nerve function, course of desease, imaging findings,surgical method and related factors became the investigative point for prognosis of cervical spondylotic myelopathy. Present viewpoint showed that the older patient, preoperative worse nerve function, longer the course of disease would result in worse outcomes. Imaging examination maybe can indicate the prognosis, but the correlation is unclear. Selection of surgical method and approach should be based on the principles of sufficient decompression, stabilize the alignment of the cervical spine, keeping backward extension of cervical spine, maintain effective decompression, preventing complications. Therefore, the treatment of cervical spondylotic myelopathy should be on the basis of pathogenic condition and imaging examination at early stage and a suitable usrgical procedure should be performed to obtain a better prognosis.
Cervical Vertebrae ; surgery ; Humans ; Magnetic Resonance Imaging ; Prognosis ; Radiography ; Spinal Cord Diseases ; diagnosis ; diagnostic imaging ; surgery ; Spondylosis ; diagnosis ; diagnostic imaging ; surgery

Adult ; Aortic Valve ; microbiology ; pathology ; Autopsy ; Brain ; microbiology ; pathology ; Endocarditis, Bacterial ; complications ; microbiology ; pathology ; Female ; Heart Ventricles ; microbiology ; pathology ; Humans ; Mitral Valve ; pathology ; Sepsis ; complications ; microbiology ; pathology ; Substance Abuse, Intravenous ; complications ; microbiology ; pathology ; Young Adult