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Neurofibromatosis type 1(NF1)is one of the most common autosomal dominant disorders. The disease is caused by mutations in the NF1 gene,which can involve multiple systems and have a variety of clinical manifestations,including café au lait macules,lisch nodules,neuroglioma,autism spectrum disorder,learning difficulties,neurofibromas,and skeletal dysplasia,et al.In previous studies on the pathogenesis of NF1,most of them have focused on the regulation of the RAS signaling pathway by neurofibromin. In recent years,researchers start exploring pathways other than RAS signaling to explore the potential functions of neurofibromin. This article reviews the research progress on the pathogenesis of NF1 in recent years,aiming to provide new ideas for treatment.
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Objective Using infrared spectroscopy to analyze three kinds of lignum Chinese medicine to provide a theoretical basis for the establishment of medicinal overall quality evaluation system. Methods Functional groups identification, Fourier Transform-infrared spectroscopy(FT-IR) and the second derivative infrared spectroscopy (SD-IR) could be applicable to analyze and identify the overall chemical composition of three kinds of lignum Chinese medicine. Results The main chemical components of Aquilariae Lignum Resinatum (ALR), Dalbergiae Odoriferae Lignum (DOL) and Sappan Lignum (SL) were cellulose, lignin and aromatics. The main chemical compositions of Sappan Lignum (SL) were cellulose, lignin and aromatics. The differences in FT-IR and SD-IR of DOL and SL indicated that they had different aromatic compounds. Conclusion Infrared spectroscopy can be used as a simple and accurate quality control method of three kinds of lignum Chinese medicine.
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Objective To provide effective reference for quality analysis of the chemical composition and extraction of astragalus separation process by comparing the extract of astragalus and it’s IR spectra. Methods The saponins and flavonoids in astragalus were firstly extracted by the method of circumfluence with ethanol as solvent and the residue of ethanol-extraction was then used to extract polysaccharides by distilled water. Fourier transform infrared spectroscopy (IR) combined with second derivative infrared spectroscopy was applied to quickly identify astragalus herbs powder, water extraction of astragalus, astragalus alcohol extraction and water extraction of the residue of ethanol-extraction. Results The powder and 70% ethanol extract, 80% ethanol extract were around at 1 735 cm-1 (carbonyl stretching vibration absorption peak) has a weak, broad absorption, while the absorption peak was less obvious in boiling water extraction. So the maln components of astragalus water extraction are polysaccharides and also contaln a small amount of water-insoluble flavonoids. Alcohol extraction malnly contalns saponins and flavonoids, and flavonoid extract volume increases with increasing alcohol concentration in a certaln range.Conclusion This method can be a quick and easy identification for astragalus and it’s extraction for its chemical composition class, and provide the basis for further research quality.