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Traditional Chinese medicine （TCM） is the treasure of the Chinese nation. As an important raw material for clinical treatment of diseases， Chinese materia medica plays an extremely important role. However， in the process of transformation from traditional wild collection of animals and plants to modern artificial cultivation and industrial production of preparations， whether the quality of Chinese materia medica is fully transferred will directly affect the quality and clinical efficacy of Chinese materia medica preparation. From the field to the sickbed， process control of quality transfer of Chinese materia medica is the key to guarantee quality and curative effect. In this paper， the whole process that affects the quality of Chinese materia medica preparations such as seed and seedling， planting and breeding， harvesting and processing， processing of decoction pieces and preparation production was analyzed. Paying attention to the whole process of quality control of Chinese materia medica is of great significance to improve the quality of Chinese materia medica preparations and promote the rapid development of TCM. Based on this， the author intended to analyze the key control links in the quality transfer process of Chinese materia medica （breeding， planting areas and field management， timely harvesting and intensive primary processing， appropriate processing， optimization of preparation technology， standardization of packaging and informationization of storage and transportation）， in order to provide reference for the design and development of Chinese materia medica preparations guided by clinical value.
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Objective To explore the association between one common variant in ABCB11-1331T ＞ C (V444A) and neonatal cholestasis.Methods One hundred and ninety-two children with neonatal cholestasis were enrolled as case group,and 196 healthy children were selected as healthy control group.The SNP site of V444A was tested by fluorescent quantitative PCR.Fisher's exact test was performed to detect the differences in allele and genotype distribution between the 2 groups.Wilcoxon rank-sum test was used to test the differences of total bilirubin,total bile acid,γ-glutamyl transpeptidase levels among the patients with different genotypes.Results TT,TC and CC genotypic distribution of V444A were not significantly different between patients and controls (P =0.530).The T allele in the case group accounted for 29.9％,in the healthy control group accounted for 26.3％,there was no significant difference between the 2 groups(OR =1.12,P =0.264).Total bilirubin,total bile acid,γ-glutamyl transpeptidase levels in patients with different genotypes of V444A were also not statistically different (all P ＞ 0.05).Conclusion Only V444A variant may have no impacts on neonatal cholestasis.
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<p><b>OBJECTIVE</b>Cleidocranial dysplasia (CCD) is a dominantly inherited skeletal dysplasia caused by mutations in the osteoblast-specific transcription factor-encoding gene, core binding factor α1 (CBFA1). Over 90 mutations in CBFA1 gene have been published to date in 500 independent cases of CCD, including missense mutations, deletions, insertions, frameshift, and splice mutations. However, mutational screening of the CBFA1 gene is still far from saturation, and more novel mutations will be identified to enrich the insights into the molecular basis for the pathogenesis of CCD. The aim of this study was to explore the clinical and image features and detect the mutations of CBFA1 gene in two CCD families.</p><p><b>METHOD</b>In this study, the clinical features were investigated in two CCD families, radiological and CT examinations regarding osseous malformation were carried out over the entire body of these patients with CCD. Blood (2 ml) was drawn from all affected individuals, unaffected family members and one hundred unrelated normal controls, Genomic DNA was extracted from whole blood with PureGene DNA extraction kit and PCR was performed with eight pairs of PCR primers for exons 0 to 7 of the CBFA1 gene. The mutations of CBFA1 gene were screened in these two CCD families.</p><p><b>RESULT</b>(1) The clinical features of patients with CCD include delayed closure of fontanelles, frontal bossing, dysplasia of clavicles, late tooth eruption, and other skeletal anomalies. X-ray and CT examination showed the bulging calvarium, patent fontanelles, wide cranial sutures, multiple Wormian bones, dental dysplasia or aplasia of clavicles. (2) Two mutations were identified, one is novel missense mutation (c.1259C > T[p.T420I]) in CBFA1 gene exon 7, other (c.577C > T[p.R193X]) was reported in Chinese cases with CCD for the first time.</p><p><b>CONCLUSION</b>(1) The clinical and image features of patients in two CCD families include delayed closure of fontanelles, frontal bossing, dysplasia of clavicles, late tooth eruption, and other skeletal anomalies. (2) The T420I and R193X mutations of CBFA1 were reported, expanding the spectrum of CBFA1 mutations causing CCD.</p>
Subject(s)
Child , Child, Preschool , Female , Humans , Male , Cleidocranial Dysplasia , Genetics , Pathology , Core Binding Factor Alpha 1 Subunit , Genetics , DNA Mutational Analysis , Exons , Mutation , Pedigree , PhenotypeABSTRACT
Objective To explore the possibility of basic fibroblast growth factor(bFGF)and epidermal growth factor(EGF)combined with striatal conditioned medium promoting the directional differentiation of rat bone marrow mesenchymal stem cells(BMMSCs)into dopaminergie neurons.Methods 1.Separation and culture of BMMSCs:BMMSCs were harvested from healthy adult Wistar rats for serial subcultivation.2.Preparation of Striatal conditioned medium:newborn Wistar rats within 24 hours were selected,and their brain tissues were removed to prepare striatal conditioned medium.3.Induced differentiation of BMMSCs:the 5th passage BMMSCs were collected and pre-induced in low glucose-Dulbecco's modified eagle medium(L-DMEM)containing bFGF and EGF.Twenty-four hours later,pre-induction liquor was replaced with striatal conditioned medium for further induced differentiation.4.Result assessment:the morphological changes of stem cells were observed under inverted phase microscope.The expression of neuron specific enolage(NSE)and tyrosine hydmxylage(TH)were identified by immunocytochemical technique.Results The cell body of rat BMMSCs contracted into round and spindle shape after induction by bFGF and EGF combined with striatal conditioned medium.Partial neuron-like cells with prominence could be found.Immunocytochemieal detection showed that the percentages of NSE and TH positive cells were(72.70±14.81)% and(34.50±15.93)%,respectively.Conclusion BMMSCs can be induced directionally into dopaminergiC neurons by bFGF and EGF combined with striatal conditioned medium in vitro.
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<p><b>BACKGROUND</b>Macrophage stimulating protein (MSP) is produced by human bone marrow endothelial cells. In this study, we sought to observe its effects on inducing the expansion of early hematopoietic progenitor cells which were cultured in a liquid culture system in the presence of the combination of stem cell factor (SCF), interleukin 3 (IL-3), interleukin 6 (IL-6), granulocyte macrophage-colony stimulating factor (GM-CSF), erythropoietin (EPO) (Cys) and MSP or of Cys and bone marrow endothelial cell conditioned medium (EC-CM).</p><p><b>METHODS</b>Human bone marrow CD34(+) cells were separated and cultured in a liquid culture system for 6 days. Granulocyte-macrophage colony forming unit (CFU-GM) and colony forming unit-granulocyte, erythrocyte, macrophage, megakaryocyte (CFU-GEMM) were employed to assay the effects of different treatment on the proliferation of hematopoeitic stem/progenitor cells. The nitroblue tetrazolium (NBT) reductive test and hoechest 33258 staining were employed to reflect the differentiation and apoptosis of the cells respectively.</p><p><b>RESULTS</b>MSP inhibited the proliferation of CFU-GM and CFU-GEMM in semi-solid culture and the inhibitory effect on CFU-GEMM was stronger than on CFU-GM. MSP inhibited the differentiation of early hematopoietic progenitor cells induced by hematopoietic stimulators. Bone marrow (BM) CFU-GEMM was 2.3-fold or 1.7-fold increase or significantly decreased in either Cys + EC-CM, Cys + MSP or Cys compared with 0 hour control in liquid culture system after 6 days.</p><p><b>CONCLUSION</b>MSP, a hematopoietic inhibitor, inhibits the differentiation of early hematopoietic progenitor cells induced by hematopoietic stimulators and makes the early hematopoietic progenitor cells expand in a liquid culture system.</p>