Pulmonary fibrosis is a group of chronic lung diseases caused by various factors and characterized by chronic inflammations,lung tissue structure damage,increase of pulmonary interstitial collagen and massive deposition of extracellular matrix (ECM). Because of its complicated etiology, there is no effective treatment currently. Recent studies showed that the activation of sphingolipids signaling and pulmonary fibrosis were closely related. This paper describes the composition and function of sphingolipids signaling pathway and its effect on fibrosis in order to provide new ideas about further study of the pathogenesis of pulmonary fibrosis and methods of prevention.

Objective To investigate the effects of Schisandrae Sphenantherae Fructus and processed with vinegar on lipid metabolism of rats with type 2 diabetes mellitus (T2DM). Methods The rat model of T2DM was induced by high fat diet plus STZ. The rats were randomly divided into blank group, model group, Schisandrae Sphenantherae Fructus high-dose group and low-dose group, and vinegar Schisandrae Sphenantherae Fructus high-dose group and low- dose group. The rats in each group were fed with the corresponding medicine for gavage for 30 d. FINS, FFA, TC, TG, HDL-C, LDL-C, and MDH, total protein content of liver tissue were detected. HE staining was used to observe the histomorphological changes of liver and pancreas in rats. Results Compared with the model group, Schisandrae Sphenantherae Fructus groups and vinegar Schisandrae Sphenantherae Fructus groups did not show obvious effects on improving FBG and FINS, but it could raise varying degrees of HDL-C and MDH, and reduce FFA, LDL-C, TC, and TG, among which vinegar Schisandrae Sphenantherae Fructus could significantly regulate metabolism in T2DM rats. Conclusion Vinegar Schisandrae Sphenantherae Fructus can enhance the lipid metabolism regulatory function of T2DM rats.

Objective To develop a more reliable blinking emergency location transmitter with longer time for aircrew in distress. Methods The new LED and new type of explosion-proof casing material (Polycarbonate) with new technologies are adopted. Results Environmental tests and performance tests were done on the new emergency location transmitter according to the environmental requirements of aeronautic products. Conclusion The new emergency location transmitter can meet the operation requirements for aircrew in distress.

Objective To assess the frequency of different subtype of spinocerebellar ataxias (SCAs) in Chinese Han population. Methods The nueleotide repeat mutations of SCA1, SCA2, SCA3/ MJD, SCA6, SCAT, SCA8, SCA10, SCA12, SCA17 and dentatorubral-pallidoluysian atrophy (DRPLA) were detected by the polymerase chain reaction (PCR), denaturing polyacrylamide gel electrophoresis (PAGE), Southern blot, recombinant DNA technology by T-vector cloning and direct sequencing technique in a cohort of 559 Mainland Chinese patients affected by spinocerebellar ataxia, including 363 families with autosomal dominant SCA (AD-SCA) and 196 sporadic cases. Results Among the 363 AD-SCA families, 15 families (4. 13%) were positive for SCA1, 26 (7. 16%) for SCA2, 187 (51.52%) for SCA3/MJD, 6 (1.65%) for SCA6, 7 (1.93%) for SCA7, 1 (0. 28%) for SCA12 and 1 (0. 28%) positive for SCA17; 120(33. 06%) were negative for all the tested SCAs. There were 2 (1.02%) SCAI, 3 (1.53%) SCA2, 15 (7. 65%) SCA3/MJD, 3 (1.53%) SCA6 and 173 (88.27%) not identified in the 196 sporadic SCA patients. None of the SCA8, SCA10 and DRPLA mutation was found. Conclusions SCA3/MJD is a substantially common subtype of AD-SCAs and sporadic SCA in Chinese Han patients with SCAs, subsequently followed by SCA2, SCA1, SCAT and SCA6; SCA12 and SCA17 are uncommon subtypes, while SCA8, SCA10, and DRPLA are rare, if not absent. SCA17 subtype was initially identified in mailand China. Some other genes might be causative in those unidentified AD-SCA pedigrees, and other etiological factors besides genetic cause might contribute for those sporadic cases.

OBJECTIVETo study the single-nucleotide substitution (c.-16C to T) of the PURATROPHIN-1 gene in spinocerebellar ataxia (SCA) patients in China.

METHODSThe single-nucleotide substitution (c.-16C to T) of the PURATROPHIN-1 gene was detected by PCR, digested with EcoN I, separated on 8% polyacrylamide gel in 68 probands of autosomal dominant SCA families and 119 sporadic SCA patients, who had been excluded CAG/CAA repeat expansion at the SCA1, 2, 3, 6, 7, 17 and dentatorubral-pallidolluysian atrophy (DRPLA) loci. The results were confirmed in four patients by direct sequencing.

RESULTSThe single-nucleotide substitution (c.-16C to T) of the PURATROPHIN-1 gene was not identified in authors' cohort.

CONCLUSIONThe mutation of c.-16C to T of the PURATROPHIN-1 gene might be rare in SCA patients in China.

Asian Continental Ancestry Group ; genetics ; Cohort Studies ; Guanine Nucleotide Exchange Factors ; genetics ; Humans ; Mutation ; Polymerase Chain Reaction ; Polymorphism, Single Nucleotide ; Spectrin ; genetics ; Spinocerebellar Ataxias ; genetics

Objective This study aims to investigate the moral disgust cognitive processing of patients with obses?sive-compulsive disorder (OCD) and its relationship with OCD symptoms. Methods Twenty-eight OCD and 30 healthy controls matched for gender, age and education completed lexical decision task, recording reaction time and accuracy of words and assessing the degree of disgust. Yale-Brown obsessive-compulsive scale (Y-BOCS) and Padua Invento?ry-Washington State University Revision (PI-WUSR) were used to assess the symptoms. Results OCD group showed significantly longer reaction time to core disgust-related words [(762.69 ± 128.25) ms vs. (648.69 ± 162.66) ms] and moral disgust-related words [(798.73 ± 115.26) ms vs. (727.00 ± 106.06) ms] than the healthy controls (P<0.05). OCD group showed significantly higher aversion degree to core disgust-related words [(6.38 ± 1.78) vs. (5.03 ± 1.64)] and moral dis?gust-related words [(7.08 ± 1.23) vs. (5.77 ± 1.44)] than control group (P<0.05). Y-BOCS total score, Y-BOCS obsessive thoughts score, Y-BOCS compulsive behavior score, total score of PI-WUSR, cleaning/pollution force factor score, hurt?ing themselves and others force factor were positively correlated with two types of disgust-related words in patients group (P<0.05). Multiple stepwise regression analysis between disgust words and Y-BOCS/PI-WUSR scores pointed that only CWCF influenced disgust degree of core disgust-related words (β=0.61, P<0.01) and moral disgust-related words (β=0.54, P<0.01), respectively. Conclusion The core disgust and moral disgust of OCD are stronger compared to controls.

Objective: As a gate-keeper enzyme link, pyruvate dehydrogenase E1 subunit alpha (PDHA1) functions as a key regulator during glycolysis and the mitochondrial citric acid cycle, which has been reported in several tumors. Nevertheless, the effects of PDHA1 on biological behaviors and metabolism remain unclear in cervical cancer (CC) cells. The study aims to explore the PDHA1 effects on glucose metabolism in CC cells and its possible mechanism. Methods: We first determined the expression levels of PDHA1 and activating protein 2 alpha (AP2α) as a PDHA1 potential transcription factor. The effects of PDHA1 in vivo were evaluated through a subcutaneous xenograft mouse model. Cell Counting Kit-8 assay, 5-ethynyl-2′-deoxyuridine (EdU) labeling assay, Transwell invasion assay, wound healing assay, Terminal deoxynucleotidyl transferase dUTP nick end labeling assay and flow cytometry were performed in CC cells. Oxygen consumption rate (OCR) levels were determined to reflect aerobic glycolysis level in gastric cancer cells. Reactive oxygen species (ROS) level was measured with 2′, 7′-dichlorofluorescein diacetate kit. The relationship between PDHA1 and AP2α was examined by conducting chromatin immunoprecipitation assay and electrophoretic mobility shift assay. Results: In CC tissues and cell lines, PDHA1 was downregulated, while AP2α was upregulated. Overexpression of PDHA1 remarkedly inhibited the proliferation, invasion and migration of CC cells, and tumor growth in vivo, as well as promoted OCR, apoptosis and ROS production. Moreover, AP2α directly bound to PDHA1 within suppressor of cytokine signaling 3 promoter region to negatively regulate PDHA1 expression level. What is more, PDHA1 knockdown could effectively reversed the AP2α silencing-mediated suppressive effects on cell proliferation, invasion, migration, and the promotive effects of AP2α knockdown on OCR, apoptosis and ROS production. Conclusions Our findings demonstrate that AP2α negatively regulated PDHA1 via binding to PDHA1 gene promoter to promote malignant CC cell behaviors, which may provide a potential approach for CC therapeutics.

OBJECTIVETo investigate the CAG trinucleotide repeat expansion in spinocerebellar ataxia (SCA) types 1, 2, 3, 6, 7, 12, and 17 from Chinese Han.

METHODSThe pathological CAG triplet repeat expansions of the SCA1, SCA2, SCA3/Machado-Joseph disease (MJD), SCA6, SCA7, SCA12 and SCA17 genes were analyzed in a cohort of 559 Mainland Chinese patients affected by spinocerebellar ataxia, including 363 probands from families with autosomal dominant SCA and 196 sporadic cases. Polymerase chain reaction, agarose gel electrophoresis, recombinant DNA technology by T-vector cloning and direct sequencing were performed to detect the CAG-repeat number of abnormal allele.

RESULTSAmong the 559 SCA patients, twenty-three were positive for SCA1, the ranges of expanded CAG repeats were from 39 to 60 (mean:51.09+/-4.88); thirty-two were positive for SCA2, the ranges of expanded CAG repeats were from 36 to 51 (mean:40.34+/-4.40); three hundred and five were positive for SCA3/MJD, the ranges of expanded CAG repeats were from 49 to 86 (mean:73.84+/-5.07); nine were positive for SCA6, the ranges of expanded CAG repeats were from 23 to 29 (mean:25.56+/-1.94); twenty-seven were positive for SCA7, the ranges of expanded CAG repeats were from 38 to 71(mean:58.22+/-10.90); three were positive for SCA12, the ranges of expanded CAG repeats were from 51 to 52 (mean:51.33+/-0.58); and finally, two were positive for SCA17, the range of expanded CAG repeats were from 53 to 55 (mean:54.00+/-1.41).

CONCLUSIONThe 39 CAG repeats of SCA1, 49 CAG repeats of SCA3 and 51 CAG repeats of SCA12 are all the shortest known causative expanded alleles, while the 86 CAG repeats of SCA3/MJD is the largest full expanded allele that has never been reported. Furthermore, it is the first report of SCA17 subtype in Mainland Chinese and first research that established the abnormal reference standard of CAG repeat number of different subtypes of SCA in Chinese Han.

Adolescent ; Adult ; Aged ; Asian Continental Ancestry Group ; ethnology ; genetics ; Ataxin-7 ; Ataxins ; Base Sequence ; Child ; Child, Preschool ; Cohort Studies ; Humans ; Male ; Middle Aged ; Molecular Sequence Data ; Nerve Tissue Proteins ; genetics ; Protein Phosphatase 2 ; genetics ; Spinocerebellar Ataxias ; ethnology ; genetics ; Trinucleotide Repeat Expansion ; Young Adult

Objective To investigate the expression of BRCA1 in familial thyroid cancer (FTC) and the relationship between BRCA1 expression and clinicopathologic features.Methods The expression of BRCA1 protein and mRNA was detected respectively by immunohistochemistry and RT-PCR in 37 FTC tissues,35 sporadic thyroid carcinoma tissues and 35 normal thyroid tissues.Results BRCA1 mRNA expression was significantly downregulated in FTC (0.210±0.025) compared with sporadic thyroid carcinoma(0.943±0.021) and normal thyroid tissues(1.001±0.087)(P＜0.01).The positive rate of BRCA1 protein expression was significantly lower in FTC(56.8％,21/37) than that in sporadic thyroid carcinoma(85.7％,30/35) and normal thyroid tissues(94.5％,33/35) (P＜0.05).There was a correlation between BRCA1 protein expression and FTC clinicopathologic features (P＜0.05).FTC patients with lower BRCA1 protein expression tended to have higher risk for bilateral carcinoma,lymph node metastasis,capsular invasion,morbidity under 30 years old and more than 2 patients in a family.Conclusions The expression of BRCA1 protein and mRNA is downregnlated in FTC.BRCA1 protein might serve as a potential biomarker for predicating biological behavior and prognosis of FTC.

The management of drug clinical trial institutions from the risk management point of view is investigated. According to the technology of risk assessment in risk management, taking the project audit as an example, basing on the basic procedures of risk identification, risk assessment and risk control, making 8 risk factors into the table of risk management for project approval of clinical trial in order to initially establish a risk management of drug clinical trial institutions. In this way, the management quality of project for drug clinical trials will be probably improved, so that the risk incidence rate will be effectively reduced in the later phase of clinical trial.