Kennedy disease,a motor neuron disease,is an adult onset form of spinal and bulbar amyotrophy characterized by the damage of low motor neuron and uncompleted androgen insensitivity syndrome.So far,there is no effective treatment.This article is intended to provide neurologist with information about Kennedy disease in epidemiology,etiology,pathogenesis,clinical feature,treatment and so on.

Background Studies showed that macophenolic acid (MPA)down-regulates and inhibits the expression and secretion of tissue growth factor and inflammatory factor,and further impacts the proliferation and inflammation process.Pterygium is an inflammatory and proliferative lesion.Whether MPA has an inhibitory effect on pterygium is unclear.Objective This study was to investigate the antifibrotic effects of macophenolic acid on pterygium fibroblasts(PFBs) in vitro and discuss its mechanism.Methods Pterygium tissue was obtained from pterygium patient during the surgery.PFBs were cultured using explants and identified with vimentin immunohistochemisty.0,0.125,0.250,0.500,1.000 μmol/L MPA were added into the culture medium,respectively,and the cells were cultured in the medium without MPA as the control group.MTT colorimetry was used to find the optimization effective concentration of MPA and evaluate their inhibitory effect on PFBs,and BrdU fluorescence staining was used to assess the growth statue of PFBs.Expressions of nuclear factor-κB(NF-κB),p65 and inhibitor of NF-κB-α(IκB-α) in the cells were detected by Western blot.Results The cells was spindle in shape 3 days after cultured and showed the vortex and radial arrangement with the positive response to vimentin.With the increase of MPA,the proliferative value of PFBs (A560)showed gradually decline,with a significant difference among the five groups (F =42.874,P＜0.01).In addition,the proliferative value of PFBs (A560) significantly lowed as the prolong of MPA active time(F=26.038,P＜0.01).BrdU fluorescence staining showed a significant decrease of DNA synthesis of PFBs with the elevation of MPA dose among the five groups(F=175.279,P＜0.05),and the A560of PFBs DNA synthesis in different concentrations of MPA groups was lower than that of the control group (all at P＜0.05).No apoptotic and necrotic cell was found after MPA action by DAPI staining.The expression level of p65 in the PFBs was 0.886±0.072 and 1.542±0.124 in the MPA group and the control group,indicating a declined value in the MPA group(P＜0.05).However,the expression value of IκB-α in the cytoplasm PFBs was significantly higher in the MPA group compared with the control group(2.141 ±0.305 vs.1.559±0.267) (P＜0.05).Conclusions MPA has an inhibitory effect on the growth of PFBs,which probably is related to the arresting of NF-κB pathway.

OBJECTIVETo investigate the effects of hypoxia on lipid metabolism in the normal human hepatic cell line L02 and to investigate the underlying molecular mechanisms.

METHODSL02 cells were exposed to hypoxic conditions (experimental groups: at 1% O2, 5% CO2, 94% N2 for 3, 6, 12, 24, or 48 hours) or normoxic conditions (control group: at 21% O2). Lipid droplet accumulation and triglyceride content were measured in each group by oil red O staining and biochemical assay, respectively. The mRNA expression levels of hypoxia-inducible factor (HIF)-2a and sterol regulatory element binding protein (SREBP)-1c were detected by reverse transcription-polymerase chain reaction. Protein expression levels of HIF-2a, adipose differentiation-related protein (ADRP), and Fas were tested by Western blot analysis.

RESULTSLipid droplet accumulation and the triglyceride content were significantly higher in the hypoxia group than the normoxia group. In addition, the hypoxia groups had significantly down-regulated mRNA expression of SREBP-1c (12h: 0.236+/-0.043, 24 h: 0.287+/-0.044, 48 h: 0.342+/-0.049 vs. normoxia: 0.503+/-0.037; F = 28.37, P less than 0.01) and FAS protein (12 h: 0.562+/-0.054, 24 h: 0.674+/-0.062, 48 h: 0.682+/-0.057 vs normoxia: 0.857+/-0.069; F = 16.08, P less than 0.01). In normoxic cells, little or no expression of HIF-2a protein was detected by Western blot. In hypoxic cells, HIF-2a protein expression peaked at 6h (0.973+/-0.067). ADRP protein expression was significantly higher in hypoxia groups than in the normoxia group (12 h: 0.319+/-0.043, 24 h: 0.732+/-0.056 and 48 h: 0.873+/-0.066 vs. 0.211+/-0.019; all, P less than 0.05.

CONCLUSIONExposure to hypoxic conditions might induce lipidosis in normal human hepatic cells by stimulating HIF-2a and ADRP expression.

Basic Helix-Loop-Helix Transcription Factors ; metabolism ; Cell Hypoxia ; Cell Line ; Down-Regulation ; Hepatocytes ; metabolism ; Humans ; Lipid Metabolism ; Membrane Proteins ; metabolism ; Oxygen ; metabolism ; Perilipin-2 ; Sterol Regulatory Element Binding Protein 1 ; metabolism

OBJECTIVETo investigate the effect of Hepatitis B Virus X Protein (HBx) on the expression of lipid metabolism-related genes and its role in pathogenesis of hepatocyte fatty degeneration.

METHODSHepatitis B Virus X gene eukaryon expression vector pIRES2-eGFP-HBx was transfected into HepG2 cells to establish HepG2/HBx cell model for HBx expression. HepG2 cells transfected with pIRES2-eGFP (HepG2/pIRES2 cell) and non-transfected were used as controls. At 24, 48 and 72 hours after transfection, the expression of green fluorescent protein (GFP) was observed by fluorescence microscope and the triglyceride(TG) content was detected. RT-PCR and Western blot were applied to detect the levels of sterol regulatory element binding protein-1 (SREBP-1), liver x receptor alpha (LXRalpha) mRNA and the levels of HBx, LXRalpha and fatty acid synthase (FAS) protein. At 24, 48 and 72 hours after transfection, the expression of GFP was found in HepG2/HBx and HepG2/pIRES2 cells, and increased gradually. The expression of HBx was detected only in HepG2/HBx cells, and was increased with time after transfection (F = 32.21, P less than 0.01). These suggested successful obtaining of HepG2-HBx cell model for HBx expression.

RESULTSAt 24h, 48h and 72h after transfection, the expression levels of LXRalpha mRNA (0.386+/-0.055, 0.505+/-0.071, 0.649+/-0.058 ) and SREBP-1 mRNA (0.395+/-0.055, 0.548+/-0.047, 0.795+/-0.058), as well as the levels of LXRalpha protein(0.178+/-0.036, 0.263+/-0.047, 0.347+/-0.058) and FAS protein(0.436+/-0.055, 0.608+/-0.053, 0.827+/-0.046) in HepG2-HBx group were dramatically higher than those in the controls at the same time points (all P less than 0.05/0.01), and were gradually increased with time (all P less than 0.05/0.01). A positive correlationship was observed between HBX protein level and the LXRalpha, SREbP-1 mRNA and LXRalpha, FAS protein levels. The difference of TG content between HepG2/HBx group and control groups was not statistically significant (P more than 0.05).

CONCLUSIONSHBx-LXRalpha-SREBP-1/FAS pathway suggested regulating transcription and expression of lipid metabolism-related genes, which might be one of the important molecular mechanism causing hepatocyte fatty degeneration.

Carcinoma, Hepatocellular ; metabolism ; Fatty Acid Synthase, Type I ; metabolism ; Hep G2 Cells ; Humans ; Lipid Metabolism ; genetics ; Liver Neoplasms ; metabolism ; Liver X Receptors ; Orphan Nuclear Receptors ; metabolism ; Sterol Regulatory Element Binding Protein 1 ; metabolism ; Trans-Activators ; genetics ; Transfection

Objective: To observe the clinical efficacy of Xingnao-Kaiqiao acupuncture("醒脑开窍"针法) on patients with cerebral infarction.Methods: Sixty-three patients with the disease were randomly divided into treatment group(n=32) treated with Xingnao-Kaiqiao acupuncture and control group(n=31) with(traditional) acupuncture.In addition,routine medicine therapies were given to the two groups(including(dehydration),(decrease) of intracranial pressure,enhancement of nerve nutrition and supportive treatment).(Xingnao-)(Kaiqiao acupuncture) was used and adjusted the number of points with different syndromes in the treatment group,main points were as follows: Neiguan(内关PC6),(Rengzhong)(人中GV26),Sanyinjiao((三阴交)SP6) and vice points were Jiquan(极泉HT1),Chize ((尺泽LU5)),Weizhong(委中BL40),Fengchi(风池GB20),Yintang (印堂EX-HN3),Shangxing((上星DU23)-through-Baihui)(百会GV20).Traditional acupuncture was used in the control group.Points at the upper limbs were Jianjing(肩井GB 21),Quchi(曲池LI 11),Waiguan(外关(S)51),Hegu(合谷LI 4) and at the lower limbs were Zusanli(足三里ST36),Yanglingquan(阳陵泉GB34),Huantiao(环跳GB30),Fenglong(丰隆ST40),Kunlun(昆仑BL60) etc..Acupuncture was given twice a day for 15 days in both groups.Before and after therapy,the hemorrheology,blood lipid,blood,urine,stool and biochemical routine examinations,white blood cell (WBC) count in(peripheral) blood and neurological deficit score(NDS) were(determined).Curative effects of two groups after treatment were observed.Results: After treatment,the total effective power was 93.75% in the treatment group,while it was 67.74% in the control group,the difference being significant (?~2=4.85,P

Objective To explore the clinical features of psoriasis vulgaris in patients with abnormal body mass index (BMI). Methods Three hundreds and ninety-seven patients with psoriasis vulgaris were investigated by questionnaire and physical examination, among them 245 patients at the progressive stage were selected for evaluating the degree of severity. The following items were compared between BMI≥25 group and BMI0.05). In the male patients, the average age in BMI≥25 group was higher than in BMI0.05). For the female patients, the subjects in BMI≥25 group had higher mean age and initial onset age, and longer diseace course than those in BMI0.05). Conclusion The psoriasis vulgaris patients with abnormal BMI always have higher average age and initial onset age, longer clinical history and severer symptoms, especially in the females.

Objective To evaluate the prevalence of metabolic syndrome(MS) in patients with psoriasis,and explore the clinical features of the patients with metabolic syndrome-associated psoriasis.Methods Two hundred and two patients with psoriasis were investigated by face-to-face questionnaire,physical and laboratory examinations.Information included age,sex,the age of onset,duration of the disease,disease severity,family history,education level,waistline,fasting plasma glucose(FPG),triglyceride(TC),high-density lipoprotein cholesterol(HDL-C) and blood pressure(BP).The diagnosis of MS was established according to the criteria of International Diabetes Federation(IDF).Results ① Of 202 patients with psoriasis,45 suffered from metabolic syndrome,accounting for 22.27%.② The patients with metabolic syndrome-associated psoriasis always had significantly higher average age and the age of onset,longer disease history and severer symptoms than those psoriasis patients without metabolic syndrome(P0.05).③ The patients with metabolic syndrome-associated psoriasis had significantly longer waistline,fasting plasma glucose,triglyceride,blood pressure and lower high-density lipoprotein cholesterol than the psoriasis patients without metabolic syndrome(P0.05).Conclusion Metabolic syndrome is highly prevalent among the patients with psoriasis,which might be associated with smoking and drinking.

The authors helpfully discuss the design idea,experimental module design,examination methods,and experiment textbook construction in experimental teaching of microbiology,and conduct further researches on the basic skill training,verifying experiment,integrative experiment,and investigative experiment in the course. This study aims to enhance effects of the experimental teaching,to cultivate high potential talents who can master essential knowledge and skills,and creatively carry out scientific research.

Objective To prepare lipid-coated ultrasound microbubbles containing 10-HCPT(HLM) and explore the antitumor effects on mice xenografed H22 solid tumor using the technique of ultrasound-mediated HLM destruction. Methods Sixty-four tumor-bearing mice were radomly divided into A and B groups. Each group was divided into four groups again and administered respectively by tail vein with HI.M, non-drug-loaded microbubbles,10-HCPT and saline once a day. Ultrasound irradiation was applied on the tumor sites immediately after injection. After 7 days of consecutive treatment, all mice in group A were sacrificed and the tumors were harvested to measure weights. The tumor inhibition rate was calculated by weights. The tumor microvessel density (MVD) was detected by immunohistochemical staining. The tumor growth curve was depicted according to volumes. The survival time of mice in group B was recorded. Results The tumor inhibition rate was the highest in HLM group while this group's MVD was the lowest. Survival time in HLM group and 10-HCPT group were obviously longer compared with the control group,while no statistic difference was observed between the two groups. There was no statistic difference between the group of non-drug-loaded microbubbles and the control group. Conclusions Ultrasound irradiation mediates HLM destruction so that the drug is released from the vihicles at the same time, which can significantly enhance the tumor inhibition effect of 10-HCPT on the H22 tumor. This technique is expected to be adopted as a novel tool for liver cancer chemotherapy.

Objective To observe the value of ultrasonography, MR and MSCT in diagnosing intravitreous autologous eyelashes. Methods Thirty-two New Zealand rabbits were averagely divided into 4 groups (A, B, C and D) randomly, and 1, 5, 10 eyelashes were implanted into vitreous body of rabbits respectively in group A, B, and C, whereas rabbits in group D (the control group) were only exposed to sham operation without implanting any eyelashes. After one week, all rabbits underwent ultrasound, MR and MSCT examination, then the specimens of vitreous bodies were obtained and observed pathologically. Results All the implanted intravitreous eyelashes were displayed with ultrasound. Intravitreous eyelashes in group A were not detected with MRI, whereas in 3 rabbits of group B and all of group C were displayed on T2WI, T2*WI and SWI sequences. MSCT detected intravitreous eyelashes in only 6 experimental animals. Conclusion Ultrasonography should be considered as the first choice for diagnosis of intravitreous autologous eyelashes. Different sequences of MRI have various advantages in diagnosing intravitreous autologous eyelashes, whereas the diagnostic value of MSCT is limited.