BACKGROUND Acromicric dysplasia(AD)is a rare skeletal dysplasia.Its incidence is<1/1000000,and only approximately 60 cases are reported worldwide.It is a disease characterized by severe short stature,short hands an...BACKGROUND Acromicric dysplasia(AD)is a rare skeletal dysplasia.Its incidence is<1/1000000,and only approximately 60 cases are reported worldwide.It is a disease characterized by severe short stature,short hands and feet,facial abnormalities,normal intelligence,and bone abnormalities.Unlike other skeletal dysplasia,AD has a mild clinical phenotype,mainly characterized by short stature.Extensive endocrine examination has not revealed a potential cause.The clinical effect of growth hormone therapy is still uncertain.CASE SUMMARY We report a clinical phenotype of AD associated with mutations in the fibrillin 1(FBN1)(OMIM 102370)gene c.5183C>T(p.Ala1728Val)in three people from a Chinese family.A 4-year-old member of the family first visited the hospital because of slow growth and short stature for 2 years,but no abnormalities were found after a series of laboratory tests,echocardiography,pituitary magnetic resonance imaging,and ophthalmological examination.Recombinant human growth hormone(rhGH)was used to treat the patient for>5 years.The efficacy of rhGH was apparent in the first year of treatment;the height increased from-3.64 standard deviation score(SDS)to-2.88 SDS,while the efficacy weakened from the second year.However,long-term follow-up is required to clarify the efficacy of rhGH.CONCLUSION FBN1-related AD has genetic heterogeneity and/or clinical variability,which brings challenges to the evaluation of clinical treatment.rhGH is effective for treatment of AD,but long-term follow-up is needed to clarify the effect.展开更多
采用高压脉冲电场(High voltage pulsed electric field,PEF)对五味子藤茎中木脂素的提取工艺进行了研究。以料液比、电场强度及脉冲数为自变量,以五味子醇甲(Schisandrol A)、五味子醇乙(Schisandrol B)、五味子甲素(Deoxyschisandrin...采用高压脉冲电场(High voltage pulsed electric field,PEF)对五味子藤茎中木脂素的提取工艺进行了研究。以料液比、电场强度及脉冲数为自变量,以五味子醇甲(Schisandrol A)、五味子醇乙(Schisandrol B)、五味子甲素(Deoxyschisandrin)、五味子乙素(Schisandrin B)等4种木脂素的得率为考察指标,在单因素试验结果的基础上,通过3因素3水平响应曲面法(Response surface methology,RSM),对五味子藤茎中4种木脂素的PEF快速提取工艺方法进行优化研究。结果表明:以80%乙醇为提取溶剂,电场强度25 k V/cm,脉冲数为8,料液比1∶30 (g∶m L)是最佳提取工艺条件,其得率为2. 28%,该提取工艺提取速度快,经济、省时,适合工艺化生产。展开更多
The present study was designed to evaluate protective activity of an ethanol extract of the stems of Schisandra chinensis(SCE) and explore its possible molecular mechanisms on acetaminophen(APAP) induced hepatotoxicit...The present study was designed to evaluate protective activity of an ethanol extract of the stems of Schisandra chinensis(SCE) and explore its possible molecular mechanisms on acetaminophen(APAP) induced hepatotoxicity in a mouse model. The results of HPLC analysis showed that the main components of SCE included schisandrol A, schisandrol B, deoxyschisandrin, schisan--drin B, and schisandrin C and their contents were 5.83, 7.11, 2.13, 4.86, 0.42 mg·g^(-1), respectively. SCE extract was given for 7 con--secutive days before a single hepatotoxic dose of APAP(250 mg·kg^(-1)) was injected to mice. Our results showed that SCE pretreatment ameliorated liver dysfunction and oxidative stress, which was evidenced by significant decreases in aspartate transaminase(AST), alanine aminotransferase(ALT), malondialdehyde(MDA) contents and elevations in reduced glutathione(GSH) and superoxide dismutase(SOD) levels. These findings were associated with the result that the SCE pretreatment significantly decreased expression levels of 4-hydroxynonenal(4-HNE) and 3-nitrotyrosine(3-NT). SCE also significantly decreased the expression levels of Bax, mitogen-activated protein kinase(MAPK), and cleaved caspase-3 by APAP exposure. Furthermore, supplementation with SCE suppressed the expression levels of inducible nitric oxide synthase(iNOS) and cyclooxygenase-2(COX-2), suggesting alleviation of inflammatory response. In summary, these findings from the present study clearly demonstrated that SCE exerted significant alleviation in APAP-induced oxidative stress, inflammation and apoptosis mainly via regulating MAPK and caspase-3 signaling pathways.展开更多
With the bioactivity-guided method,a new flavanone glycoside,together with nine known flavonoids were isolated from 50% aqueous ethanol of litchi（Litchi chinensis Sonn.） seeds.The chemical structure of the new compo...With the bioactivity-guided method,a new flavanone glycoside,together with nine known flavonoids were isolated from 50% aqueous ethanol of litchi（Litchi chinensis Sonn.） seeds.The chemical structure of the new compound was elucidated via 1D and 2D nuclear magnetic resonance（NMR） techniques and mass spectrometry to be （2S）-pinocembrin-7-O-（6＂-O-α-L-arabinosyl-β-D-glucopyranoside）（1）,and the nine known compounds were determined to be quercetin（2）,phlorhizin（3）,pinocembrin-7-O-glucoside（4）,kaempferol-7-O-β-D-glucopyranoside（5）,onychin（6）,nairutin（7）,narcissin（8）,pinocembrin-7-O-[（6＂-O-β-D-glucopyranoside）-β-D-glucopyranoside]（9） and pinocembrin-7-O-[（2＂,6＂-di-O-α-L-rhamnopyranosyl）-β-D-glucopyranoside]（10）.Some of the isolated flavonoids were tested for their inhibitory effects on α-glucosidase.And compounds 2 and 3 showed stronger inhibitory activity than positive control.展开更多
By choosing neuroimmunophilin FKBP12 as a therapeutical target, we have attempted to discover a new structural drug for treating neurodegenerative disease. This drug should possess neurotrophic activity and not affect...By choosing neuroimmunophilin FKBP12 as a therapeutical target, we have attempted to discover a new structural drug for treating neurodegenerative disease. This drug should possess neurotrophic activity and not affect the immune system. Based on the crystal structure of FKBP12, FK506 and Calcineurin complex, a series of small organic molecules were designed. These molecules were to have the ability of binding to FKBP12 in a virtual screening. By using a solution parallel synthetic method, these compounds were synthesized. The neuroprotective and neuroregenerative activities of these compounds were evaluated by binding assays, PC12 cells survival and neurite outgrowth model, chick dorsal root ganglion cultures (DRG) and 6-OHDA lesioned mice sympathetic nerve endings model. The evaluation results of these compounds showed that compound N308 has great promise as a candidate for a neuroprotective and neuroregenerative agent.展开更多
文摘BACKGROUND Acromicric dysplasia(AD)is a rare skeletal dysplasia.Its incidence is<1/1000000,and only approximately 60 cases are reported worldwide.It is a disease characterized by severe short stature,short hands and feet,facial abnormalities,normal intelligence,and bone abnormalities.Unlike other skeletal dysplasia,AD has a mild clinical phenotype,mainly characterized by short stature.Extensive endocrine examination has not revealed a potential cause.The clinical effect of growth hormone therapy is still uncertain.CASE SUMMARY We report a clinical phenotype of AD associated with mutations in the fibrillin 1(FBN1)(OMIM 102370)gene c.5183C>T(p.Ala1728Val)in three people from a Chinese family.A 4-year-old member of the family first visited the hospital because of slow growth and short stature for 2 years,but no abnormalities were found after a series of laboratory tests,echocardiography,pituitary magnetic resonance imaging,and ophthalmological examination.Recombinant human growth hormone(rhGH)was used to treat the patient for>5 years.The efficacy of rhGH was apparent in the first year of treatment;the height increased from-3.64 standard deviation score(SDS)to-2.88 SDS,while the efficacy weakened from the second year.However,long-term follow-up is required to clarify the efficacy of rhGH.CONCLUSION FBN1-related AD has genetic heterogeneity and/or clinical variability,which brings challenges to the evaluation of clinical treatment.rhGH is effective for treatment of AD,but long-term follow-up is needed to clarify the effect.
文摘采用高压脉冲电场(High voltage pulsed electric field,PEF)对五味子藤茎中木脂素的提取工艺进行了研究。以料液比、电场强度及脉冲数为自变量,以五味子醇甲(Schisandrol A)、五味子醇乙(Schisandrol B)、五味子甲素(Deoxyschisandrin)、五味子乙素(Schisandrin B)等4种木脂素的得率为考察指标,在单因素试验结果的基础上,通过3因素3水平响应曲面法(Response surface methology,RSM),对五味子藤茎中4种木脂素的PEF快速提取工艺方法进行优化研究。结果表明:以80%乙醇为提取溶剂,电场强度25 k V/cm,脉冲数为8,料液比1∶30 (g∶m L)是最佳提取工艺条件,其得率为2. 28%,该提取工艺提取速度快,经济、省时,适合工艺化生产。
基金supported by the grants of Scientific Research Foundation for the Returned Overseas Chinese Scholars(Jilin Province,2015)Jilin Science&Technology Development Plan(No.20160209008YY)the Program for the Young Top-notch and Innovative Talents of Jilin Agricultural University(2016-2018)
文摘The present study was designed to evaluate protective activity of an ethanol extract of the stems of Schisandra chinensis(SCE) and explore its possible molecular mechanisms on acetaminophen(APAP) induced hepatotoxicity in a mouse model. The results of HPLC analysis showed that the main components of SCE included schisandrol A, schisandrol B, deoxyschisandrin, schisan--drin B, and schisandrin C and their contents were 5.83, 7.11, 2.13, 4.86, 0.42 mg·g^(-1), respectively. SCE extract was given for 7 con--secutive days before a single hepatotoxic dose of APAP(250 mg·kg^(-1)) was injected to mice. Our results showed that SCE pretreatment ameliorated liver dysfunction and oxidative stress, which was evidenced by significant decreases in aspartate transaminase(AST), alanine aminotransferase(ALT), malondialdehyde(MDA) contents and elevations in reduced glutathione(GSH) and superoxide dismutase(SOD) levels. These findings were associated with the result that the SCE pretreatment significantly decreased expression levels of 4-hydroxynonenal(4-HNE) and 3-nitrotyrosine(3-NT). SCE also significantly decreased the expression levels of Bax, mitogen-activated protein kinase(MAPK), and cleaved caspase-3 by APAP exposure. Furthermore, supplementation with SCE suppressed the expression levels of inducible nitric oxide synthase(iNOS) and cyclooxygenase-2(COX-2), suggesting alleviation of inflammatory response. In summary, these findings from the present study clearly demonstrated that SCE exerted significant alleviation in APAP-induced oxidative stress, inflammation and apoptosis mainly via regulating MAPK and caspase-3 signaling pathways.
基金Supported by the National Natural Science Foundation of China（No.30873370） and the Doctor Fund of Jilin Agricultural University of China（No.201210）.
文摘With the bioactivity-guided method,a new flavanone glycoside,together with nine known flavonoids were isolated from 50% aqueous ethanol of litchi（Litchi chinensis Sonn.） seeds.The chemical structure of the new compound was elucidated via 1D and 2D nuclear magnetic resonance（NMR） techniques and mass spectrometry to be （2S）-pinocembrin-7-O-（6＂-O-α-L-arabinosyl-β-D-glucopyranoside）（1）,and the nine known compounds were determined to be quercetin（2）,phlorhizin（3）,pinocembrin-7-O-glucoside（4）,kaempferol-7-O-β-D-glucopyranoside（5）,onychin（6）,nairutin（7）,narcissin（8）,pinocembrin-7-O-[（6＂-O-β-D-glucopyranoside）-β-D-glucopyranoside]（9） and pinocembrin-7-O-[（2＂,6＂-di-O-α-L-rhamnopyranosyl）-β-D-glucopyranoside]（10）.Some of the isolated flavonoids were tested for their inhibitory effects on α-glucosidase.And compounds 2 and 3 showed stronger inhibitory activity than positive control.
基金Supported by the National Basic Research Program of China (Grant No. G1998051107)Hi-tech Research and Development Program of China (Grant No. 2002AA233051)
文摘By choosing neuroimmunophilin FKBP12 as a therapeutical target, we have attempted to discover a new structural drug for treating neurodegenerative disease. This drug should possess neurotrophic activity and not affect the immune system. Based on the crystal structure of FKBP12, FK506 and Calcineurin complex, a series of small organic molecules were designed. These molecules were to have the ability of binding to FKBP12 in a virtual screening. By using a solution parallel synthetic method, these compounds were synthesized. The neuroprotective and neuroregenerative activities of these compounds were evaluated by binding assays, PC12 cells survival and neurite outgrowth model, chick dorsal root ganglion cultures (DRG) and 6-OHDA lesioned mice sympathetic nerve endings model. The evaluation results of these compounds showed that compound N308 has great promise as a candidate for a neuroprotective and neuroregenerative agent.
