ObjectiveTo develop three animal models to mimic muscle tremors of Parkinson's disease and observe effects of Chinese herb compounds Jian-Xing on these models.MethodsPure muscle tremor mice models were developed by single intraperitoneal injection of arecoline or oxotremorine. The mitochondria damaged model was developed by intraperitoneal injection of MPTP(1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine) 20mg/kg for 8 days. Duration time of tremoring was recorded. The climbing time and spontaneous movements of MPTP mice were recorded. HPLC was used to detect the content of dopamine and it's metabolites in MPTP mice.ResultsAfter injection of oxotremorine or arecoline,model mice exhibited significant tremoring, duration time of tremor of mice in Jian-Xing group shortened significantly. As to MPTP model mice, climbing time of model mice prolonged, times of spontaneous movements of model mice decreased and the content of dopamine in striaturn decreased compared with control group. Climbing time of mice in Jian-Xing group shortened, spontaneous movements increased and content of dopamine increased distinctively. ConclusionOxotremorine, arecoline and MPTP all can produce tremor animal models. Chinese herb compounds Jian-Xing can shorten the duration time of tremor.As to MPTP model, Jian-Xing can shorten the climbing time of model mice, increase the spontaneous movements and increase the content of dopamine in striaturn.

ObjectiveTo investigate the role of mitochondrial dysfunction in neurodegenerative diseases and the pharmacological effects of Shen-wu Capsule.MethodsSD rats were divided into 6 groups with 10 rats each:model group was developed by peritoneal injection of 3-nitropropionic acid(3-NPA) 20mg/kg, once every another day. Haloferidoli was used as the positive drug (given to positive control group from 10μg/100g/day to 100μg/100g/day, during modeling). Low dose of Shen-wu Capsule is 0.45g/kg; middle dose of Shen-wu Capsule is 0.9g/kg; high dose of Shen-wu Capsule is 1.8g/kg (once a day for 25days). The rats in normal control group were peritoneally injected with saline and were fed with water.The open field test was used to test the space recognition ability and excitability of CNS of rats. The climbing test was used to test strength of muscles.The content of dopamine(DA) and it's metabolite dihydroxyphenylacetic acid (DOPAC),5-HT in stratium of all groups were detected by high performance liquid chromatograph (HPLC-ECD).ResultsThe 3-NPA model rats showed decrease of activity, rigidity and stagger just like clinical patients with Huntington's diseases. In open field test all indexes of 3-NPA model rats are decreased (P<0.05), while Shen-wu Capsule low dose and high dose can improve all the indexes. There is no difference in strength of muscles among all groups. The content of DA,DOPAC and 5-hydroxytryptamine (5-HT) were distinctly decreased in 3-NPA model rats(P<0.01); rats in three groups of Shen-wu Capsule and positive drug control groups showed increased content of these neurotransmitter(P<0.01). Conclusions Rats injected with 3-NPA can mimic the abnormal behaviors and changes of neurotransmitter in stratium of Huntington's patients, Shen-wu Capsule can ameliorate the behavior of model animals and improve the content of DA,DOPAC and 5-HT in stratium.

Objective To study effect of excessive iodine intake on sodium-iodide symporter(NIS)mRNA and protein expression of breast in lactating rats.Methods60 Wistar rats,having been weaned for one month,were randomly divided into three groups according to their body weights,I.e,①normal iodine(NI,30 rats);②ten fold high iodine(10 HI,15 rats);③one hundred fold high iodine(100 HI,15 rats).Eating food containing iodine of 300μg/L and drinking water of iodine at 5,1845,20 295μg/L,respectively.After fed for 3 months,the rats mated and had offspring,and urine and milk iodine of lactating rats were determined by As-Ce-catalytic spectrophotometric method.Their marmnary glands were sampled at lactation day 10.Then NIS mRNA expression by RT-PCR was determined and NIS protein by immunohistochemistry(SABC)was observed.Results The urine iodine of 10 HI group(3597.5μg/L)and 100HI group(25 404.3μg/L)increased obviously compared with that of NI group(344.7μg/L).The milk iodine of 10HI group(27.1×103μg/L)and 100HI group(191.0×1μg/L)was higher than that of NI group(6.0×103μg/L),but the increased fold of milk iodine was not paralleled with that of urine iodine.Difference of NIS mRNA expression was significant(F=24.19,P＜0.01)among the groups,and the NIS mRNA expression in 10HI(1.250±0.034)and 100HI(1.272±0.039)group were less than that in NI (1.532±0.044)group(P＜0.01).The breast NIS mRNA expression in lactating rats(1.532±0.044)was significantly higher than that in unlactating rats(0.879±0.018,P＜0.01).With the increasing iodine uptake,NIS protein expression decreased.Conclusions The NIS mRNA and protein in rat breasts is down-regulated by excessive iodine intake.So increasing extent of milk iodine concentration is inhibited,which is important to prevent off-spring from getting excessive iodine intake from parental generation.

Adult ; Benzene ; poisoning ; Chronic Disease ; Cytochrome P-450 CYP2E1 ; genetics ; Female ; Humans ; Leukocyte Count ; Leukopenia ; chemically induced ; Male ; Middle Aged ; Polymorphism, Genetic

BACKGROUNDObstructive sleep apnea is strongly associated with obesity, particularly abdominal obesity common in centrally obese males. Previous studies have demonstrated that intra-abdominal pressure (IAP) is increased in morbid obesity, and tracheal traction forces may influence pharyngeal airway collapsibility. This study aimed to investigate that whether IAP plays a role in the mechanism of upper airway (UA) collapsibility via IAP-related caudal tracheal traction.

METHODSAn abdominal wall lifting (AWL) system and graded CO2pneumoperitoneum pressure was applied to four supine, anesthetized Guizhou miniature pigs and its effects on tracheal displacement (TD) and airflow dynamics of UA were studied. Individual run data in 3 min obtained before and after AWL and obtained before and after graded pneumoperitoneum pressure were analyzed. Differences between baseline and AWL/graded pneumoperitoneum pressure data of each pig were examined using a Student's t-test or analysis of variance.

RESULTSApplication of AWL resulted in decreased IAP and significant caudal TD. The average displacement amplitude was 0.44 mm (P < 0.001). There were three subjects showed increased tidal volume (TV) (P < 0.01) and peak inspiratory airflow (P < 0.01); however, the change of flow limitation inspiratory UA resistance (Rua) was not significant. Experimental increased IAP by pneumoperitoneum resulted in significant cranial TD. The average displacement amplitude was 1.07 mm (P < 0.001) when IAP was 25 cmH2O compared to baseline. There were three subjects showed reduced Rua while the TV increased (P < 0.01). There was one subject had decreased TV and elevated Rua (P < 0.001).

CONCLUSIONSDecreased IAP significantly increased caudal TD, and elevated IAP significantly increased cranial TD. However, the mechanism of UA collapsibility appears primarily mediated by changes in lung volume rather than tracheal traction effect. TV plays an independent role in the mechanism of UA collapsibility.

Airway Resistance ; physiology ; Animals ; Female ; Lung Volume Measurements ; Obesity, Morbid ; physiopathology ; Sleep Apnea, Obstructive ; physiopathology ; Swine ; Tidal Volume ; physiology ; Trachea ; physiology

OBJECTIVETo evaluate the mechanical load of retropalatal airway in obstructive sleep apnea patients, and to investigate the contributions of pharyngeal anatomy to upper airway collapsibility.

METHODSStatic mechanical load of transpalatal pharynx was determined by opening pressure (Popen) of the segment during general anesthesia in 30 patients and 14 controls. Size of pharynx was measured while intraluminal pressure was controlled at 3-20 cm H2O (1 cm H2O = 0.09806 kPa) and the minimal intraluminal pressure that needed to compensate for the mechanical load of a retropalatal segment was determined.

RESULTSPharyngeal cavity collapse at the level of the hard palate was observed in only one of the 30 subjects (3.3%), and in none of the 14 controls. At tongue base level, 23 subjects had a Popen > 0 cm H2O (76.7%) while in 7 of the controls (50.0%) had a Popen > 0 cm H2O. And at the level of the uvual and soft palate, pharyngeal collapses was observed in all subjects except in 9 of the controls (64.3%). The median of Popen was 8.3 [5.9;11.5] cm H2O in the patients group and was 2.7 [-3.9;6.0] cm H2O in the control group. Differences of Popen were significant between patients and controls (U = 58.500, P = 0.000). The correlation between Popen and AHI was also significant at 0.05 level (r = 0.377, P = 0.044).

CONCLUSIONSPatients with sleep apnea have more collapsible passive upper airway than controls. Retropalatal and retroglossal airway are the most collapsible segments and positive pressures are needed to compensate for the mechanical loads.

Adult ; Case-Control Studies ; Female ; Humans ; Middle Aged ; Muscle Relaxation ; Palate, Soft ; anatomy & histology ; physiology ; physiopathology ; Pharyngeal Muscles ; physiology ; Pressure ; Sleep Apnea, Obstructive ; etiology ; pathology ; physiopathology ; Tongue ; anatomy & histology ; physiology ; Young Adult

Objective Take alkaline ashing method as golden standard to explore the accuracy of chloric acid digestion method in determination of human milk iodine. Methods Sixty one breast milk samples collected in Hexi district of Tianjin was measured by the method for determination of iodine in foodstuff by As3+-Ce4+ catalytic spectrophotometry (referred to as the alkaline ashing method) published in 2008 and the method for determination of iodine in urine by As3+-Ce4+ catalytic spectrophotometry(referred to as acid digestion) published in 1999, respectively. were highly correlated(r = 0.960, t = 26.3, P < 0.01), and the regression equation was (Y) = - 28.1 + 0.808X, in which X was independent variable, that is the results of alkaline ashing method; (Y) was dependent variable, that is the estimated data of chloric acid digestion method. The average difference of the results measured by the two methods was 68.3 μg/L, and the results from chloric acid digestion was 38.9% which lower than that of alkaline samples were diluted by 3,4 and 5-fold and then digested by chloric acid, the liquid clarification rates were 80.3% ashing and chloric acid digestion method were, respectively, 165.4, 110.0 μg/L. Conclusions Compared with alkaline ashing method, the results determined by chloric acid digestion method are significantly lower. It is suggested that there are systemic errors in chloric acid digestion method, which means that alkaline ashing method can not be replaced by the chloric acid digestion method.

A(p.G888S)were detected in POLG1 gene.Sequence analysis of parental blood DNA revealed that her father carried L83P and her mother carried G888S.Conclusions The characteristics of clinical manifestation,electrophysiology,pathology and POLG1 gene mutation of the patient were highly consistent with Alpers syndrome.The prominent white matter change and increased immunological factors in CSF were first reported in Alpers syndrome.Alpers syndrome should be considered for those patients whose liver function were severely impaired after exposure to valproic acid.

This study was aimed to evaluate the impact of regular donating platelets on serum ferritin (SF) of donors. A total of 93 male blood donors including 24 initial plateletpheresis donors and 69 regular plateletpheresis donors were selected randomly. Their SF level was measured by ELISA. The results showed that the SF level of initial plateletpheresis donors and regular plateletpheresis donors were 91.08 ± 23.38 µg/L and 57.16 ± 35.48 µg/L respectively, and all were in normal levels, but there was significant difference between the 2 groups (p < 0.05). The SF level decreased when the donation frequency increased, there were no significant differences between the groups with different donation frequency. Correlation with lifetime donations of platelets was not found. It is concluded that regular plateletpheresis donors may have lower SF level.
Adult ; Blood Donors ; Ferritins ; blood ; Humans ; Iron ; blood ; Male ; Middle Aged ; Platelet Count ; Plateletpheresis ; Serum ; chemistry ; Young Adult

OBJECTIVEPelizaeus-Merzbacher disease (PMD) is a rare X-linked recessive leukoencephalopathy. Few reports of PMD patients without genetic confirmation have been published in the mainland of China. The clinical and genetic features of a family with PMD were analyzed, which may contribute to definite diagnosis, genetic counseling and prenatal diagnosis of this rare hereditary disease in China.

METHODSClinical data of the proband and other family members as well as 14 DNA samples were collected. Clinical features including symptoms, signs and cranial MRI were analyzed. Multiplex ligation-dependent probe amplification (MLPA) assays were performed to detect PLP1 duplication, which helps identify the type of PLP1 mutation in this family and the genotype-phenotype correlations.

RESULTS(1) The proband and the other 3 male patients in the family presented with nystagmus, motor retardation followed by regression. The cranial MRI of proband showed evidence of poor myelination with diffused high signal in white matter region on T2-weighed image and reduced amount of white matter in volume, which is consistent with the typical features of cranial MRI in PMD. (2) PLP1duplication was identified in the proband. Combined with the clinical features of the proband and other patients in this family, the diagnosis of classic form of PMD was confirmed. Another 3 females with normal phenotype in the family were proved to be carriers of PLP1duplication.

CONCLUSIONS(1) The Classic form of PMD in this pedigree is resulted from the PLP1 duplication, which is consistent with the previously reported genotype-phenotype correlations; (2) The results serve as an evidence for reliable genetic counseling and prenatal diagnosis for this family. (3) MLPA, which is a newly developed method, is a rapid and reliable technique to detect the whole gene duplication of PLP1.

Adult ; DNA Probes ; Genes ; Genetic Association Studies ; Humans ; Infant ; Male ; Mutation ; Myelin Proteolipid Protein ; genetics ; Pedigree ; Pelizaeus-Merzbacher Disease ; genetics ; Phenotype