Objective To explore the clinical features and SQSTM1/p62 gene mutations in Chinese Han patients with familial amyotrophic lateral sclerosis linked superoxide dismutase 1 (SOD1) mutation (FALS-SOD1).Methods A total of 13 FALS-SOD1 probands and 100 healthy controls were studied,with DNA extracted from the peripheral blood.Sequencing was carried out at 8 exons,intron-exon boundaries and promoter region (2-kb upstream from the coding sequence) of SQSM1/p62.Clinical data were collected and all patients were followed-up.Phenotype-genotype relationship was analyzed.Results The insertion of T was found in intron 5 of SQSTM1/p62 gene [+ 1 insert T (TT ＞ TG)] in a FALS-SOD1 G16A male proband,with limbs as the symptom onset and faster disease progression than the other two SOD1 G16A probands without SQSTM1/p62 gene mutation.Conclusions The insertion of T in the intron 5 of SQSTM1/ p62 gene may promote the ALS progression by damaging p62 function in the FALS-SOD1 G16A proband.

ADAM9, a member of ADAM family, plays an important role in development of many human diseases especially neoplasms as its metaUoproteinases activity and the ability of combining with integrins. This might help us find new therapeutic targetpoints and therefore offer new therapeutic approaches.

The present valuation of experimental teaching effect can not adapt to the needs of the experimental reform and develpoment due to its deficiency. After setting up the experimental curriculum,A Multivariate method has been developed to evaluate students’ experimental ability from their innovating,operating,expressing ability and scientific quality.

Objective:To investigate microRNA-29b ( miR-29b) expression in cerebral cortex , spinal cord, fore limb muscle, and serum of SOD1-G93A amyotrophic lateral sclerosis ( ALS) mice, and to identify the biomarker and to assess diagnostic values for ALS .Methods:Cerebral cortex , spinal cord , fore limb muscle and serum from 16 SOD1-G93 A ALS mice and 16 wild-type mice were taken and then microRNA extracted , detecting the expression of miR-29 b by real-time quantitative polymerase chain re-action ( RT-qPCR ) .The diagnostic performance of miR-29b for ALS was estimated by the receiver operating characteristic ( ROC ) curve . Results: The results from the validation indicated that the differences in miR-29b between the cerebral cortex of SOD1-G93A ALS and the healthy control subjects were statistically significant (P=0.001).Meanwhile, the expressions 8, 12, and 16 weeks later were higher than those of the controls ( ALS vs.Control: 8 weeks, P=0.044; 12 weeks, P=0.018; 16 weeks, P=0.045).When the relative expression level of miR-29b was used to diagnose ALS in SOD1-G93A ALS mice, the area under the ROC (area under the curve, AUC) was 0.885, if the diagnostic threshold was set at 0.185 6, the sensitivity and specificity were 92.9%and 71.4%.Conclusion:MiR-29 b may act as medical monitoring indices of ALS in early time .

Objective To investigate the information of outpatients with acute and chronic insomnia in the department of neurology in the general hospital.Methods In the case-control study and epidemiological investigation, the sleeping information of the outpatients in Department of Neurology of Peking University Third Hospital were collected according to classification and diagnostic criteria of mental disorder in American from October 2004 to December 2004. Multiple Logistic regression was performed to analyze the associated factors for insomnia.Results Total 925 cases were investigated and divided into acute insomnia group(149 cases), chronic insomnia group (361 cases) and control group (415 cases). After adjustment for major confounders including gender, smoking, coronary artery disease, hyperlipoidemia, education and occupation, it showed that:(1) Female (OR=2.02, P=0.001), hyperplasia of prostate (OR=1.94, P=0.036), anxiety (OR=3.29, P=0.000), depression (OR=2.48, P=0.000) and leg-moving (OR=1.78, P=0.019) increased the risk of acute insomnia significantly. (2) Female(OR=2.13, P=0.000), education (OR=2.05, P=0.005;OR=2.07, P=0.003), hyperplasia of prostate (OR=1.71, P=0.023), anxiety (OR=7.42, P=0.000) and depression (OR=4.73, P=0.000) increased the risk of chronic insomnia significantly.Conclusions Female, hyperplasia of prostate, anxiety and depression are associated with acute and chronic insomnia.

Objective: High tibial osteotomy treated medial compartment serious osteoarthritis of the knee with varus deformity,and relieving arthritic pain,correcting deformity and normal femoraltibial axial alignment.Methods:From May 1992 to February 1998,18 cases(21 knees) were operated on,We chose fit patients and planed osteotomy angle.Results:All cases were followed up for an average 3.2 years,according to Coventry's rating system,81% of the whole series had a good or excellent results.Conclusion:It increases the medial joint space width and corrects femoraltibial axial alignment,allows for healing of the articular cartilage.Satisfactory results were obtained.

Objective To observe the effect of gene therapy for Parkinson disease by triple transduction with dopamine-biosynthesizing enzyme genes. Methods We constructed high titer purified adeno-associated virus (AAV) vectors containing cDNAs encoding human tyrosine hydroxylase (TH), aromatic L-amino acid decarboxylase (AADC) and GTP cyclohydrolase I (GCH) respectively. AAV-TH, AAV-AADC and AAV-GCH were stereotaxically injected into the denervated striatum of Parkinsonian rat; the expression of TH, AADC or GCH was assayed by using immunohistochemical staining; the rotational behavior was assayed for 1 year.Results The immunohistochemical staining and behavioral testing showed that all of TH, AADC and GCH were expressed stably in the striatum, and the rats treated with AAV-TH, AAV-AADC and AAV-GCH showed more remarkable decrease in rotation rate persisted for at least 1 year, than those treated with AAV-TH and AAV-AADC(P

In this study the nutritive value of 4 kinds of weaning foods containing soyflour were measured by chemical and animals experiments. The 4 experimental weaning foods were:1.This formula was developed by the Institute of Health collaborating the First Food Factory of Beijing. It was produced by the Factory and the trade name is "Baobao Le" The soybean was processed by the infra-red light.2.This formula was recommended by UNICEF (Formula No. 5). Soybean flour was treatded with the extruded method.3.This formula was the same as No. 2 (UNICEF Formula No. 5). In which the soybean was wet-heat treated. No. 2 and 3 samples were produced by Wu Xi Light Industry College.4.This formula was developed by the Institute of Shanghai Food Industry as formula D. Puffed soybean flour was used.The 2 control foods were:5.This formula was based on formula "5410". The sample was made by the First Food Factory of Beijing (trade name, "Nutritive Baby Powder").6.Infant Milk Powder, produced by the Child Milk Factory of Shuang Cheng Xian, Hei Long Jiang Province, cow's milk powder was used as the main source of protein. The protein content of the products were:No. 1 and 4, 19.0%, equal to the protein requirement for weaning foods; No.2 and 3, 15.8%, lower than No.1 and 4; Infant Milk Powder, 15.1%; Nutritive Baby Powder, 17.0%.From the results of the animal experiment on weaning rats with respect to growth, after 9 weeks of feeding, the average body weight of control group 5 and 6 increased by 185.8 and 180.5g respectively, which were significantly higher than 146.7 and 150.4g of the experimental groups No. 1 and 4. While the increased body weight of No. 1 and 4 were higher than group 2 and 3 (79.8g and 113.1g respectively). The increase of body lengths were of the same order of the 6 groups as that in body weight.Blood hemoglobin values were all normal (control groups 13.4 and 13.9g per 100 ml; experimental groups 13.9, 14.2, 13.0 and 13.0g).The weight of pancreas in the 4 experimental groups were all normal. It showed that the activity of trypsin inhibitor had been destroyed. Weights of thyroid of the rats were all within the normal range except group 1,2,3, which were a little bit heavier. Potassium iodide was.added into formula 1,2 and 3, to prevent hyperthyroidism but since potassium iodide is easily to evaporate, so it would be perferable to use potassium iodate instead.The above results indicated that the growth promoting effect of the 4 weaning foods is lower than the two baby powder, but they are good weaning foods for baby after six months. "Baobao Le" and formula No 6 are much better.

The significance of the blood glutathion reductase activity coeff i-.cient (BGR-AC) as the guide line to assess riboflavin status in 28 radar operators was studied by comparing with the amount of their urinary excreted riboflavin.The 28 subjects were randomized into 4 groups and the riboflavin intake was 0.53.mg,1.0 mg,2.0 mg,and 3.0 mg respectively.It was found that there was a significant negative correlation between BGR-AC and urinary excretion of riboflavin,and with the incrase of riboflavin intake,there was a significant decrease of BGR-AC in Groups Ⅰ,Ⅱ,and Ⅲ though the difference between Group Ⅲ and Group Ⅳ was insigificant.On the basis of the BGR-AC values of the 4 groups,an exponential equation was established which tells the riboflavin requirement of radar operators.It is defined that when BGR-AC is smaller than equal to 1.24,it is normal and when it is larger than 1.24,it means abnormal riboflavin status of the individual.