目的探讨Ⅰ、Ⅲ、Ⅴ型胶原(Col Ⅰ、Ⅲ、Ⅴ)在系统性硬化症(SSc)患者皮损中的表达、分布及其在发病机制中的作用。方法采用免疫组化SP法检测36例SSc患者(轻度纤维化9例,中度14例,重度13例)皮损中Ⅰ、Ⅲ、Ⅴ型胶原α1链〔α1(Ⅰ)、α1(Ⅲ...目的探讨Ⅰ、Ⅲ、Ⅴ型胶原(Col Ⅰ、Ⅲ、Ⅴ)在系统性硬化症(SSc)患者皮损中的表达、分布及其在发病机制中的作用。方法采用免疫组化SP法检测36例SSc患者(轻度纤维化9例,中度14例,重度13例)皮损中Ⅰ、Ⅲ、Ⅴ型胶原α1链〔α1(Ⅰ)、α1(Ⅲ)、α1(Ⅴ)〕的表达(以平均光密度值为半定量检测指标),并以6例正常皮肤作为对照。结果3种胶原在SSc皮损真皮呈弥漫性分布,在网状层可见深染的α1(Ⅰ)和α1(Ⅲ)团块或条带,α1(Ⅴ)分布则较均匀,在小血管和皮肤附属器周围较多;从对照到SSc轻度、中度、重度纤维化组,α1(Ⅰ)、α1(Ⅲ)和α1(Ⅴ)含量在真皮、小血管和附属器及其周围组织均呈逐渐升高趋势(P均<0.05);在SSc皮损真皮、小血管和附属器及其周围组织中,α1(Ⅴ)在早期即明显升高(80.07±1.80 vs 73.56±2.48,94.23±1.53 vs 85.73±1.70,94.06±1.64 vs 84.65±1.65,P均<0.01),持续整个纤维化过程且增幅较大,α1(Ⅰ)和α1(Ⅲ)升高则较迟,在中、后期显著升高。结论α1(Ⅴ)先于α1(Ⅰ)、α1(Ⅲ)在SSc早期即开始大量沉积,并持续整个纤维化过程;其含量改变始于真皮小血管和附属器及其周围组织,可能参与SSc血管炎的发生。展开更多
The crystal structure of the title compound, [enH2][Fe{MoⅤ6O12(OH)3(HPO4)- (H2PO4)3}2]6en6H2O (en = H2NCH2CH2NH2), hydrothermally synthesized from a mixture of Na2MoO42H2O, Fe2(SO4)3, H3PO4, H2N(CH2)2NH2 and water, h...The crystal structure of the title compound, [enH2][Fe{MoⅤ6O12(OH)3(HPO4)- (H2PO4)3}2]6en6H2O (en = H2NCH2CH2NH2), hydrothermally synthesized from a mixture of Na2MoO42H2O, Fe2(SO4)3, H3PO4, H2N(CH2)2NH2 and water, has been determined by single- crystal X-ray diffraction. The crystal is of triclinic, space group P?with a = 11.9014(1), b = 13.4246(2), c = 13.8719(2) , a = 87.465(1), b = 69.981(1), g = 64.960(1)? V = 1873.46(4) 3, Z = 1, Mr = 2997.89, F(000) = 1466, m = 2.427 mm-1 and Dc = 2.657 g/cm3. The final R = 0.0404 for 5570 observed reflections (I > 2s(I)). The structural analysis reveals that each cluster anion contains two coplanar {Mo6} rings of six edge-sharing Mo(O5OH) octahedra, and the two {Mo6} rings are linked together through one octahedral FeⅡ ion to generate a sandwich-type cluster with rigorous () symmetry. Moreover, these clusters are further linked into a three-dimensional frame- work by hydrogen bonds.展开更多
文摘目的探讨Ⅰ、Ⅲ、Ⅴ型胶原(Col Ⅰ、Ⅲ、Ⅴ)在系统性硬化症(SSc)患者皮损中的表达、分布及其在发病机制中的作用。方法采用免疫组化SP法检测36例SSc患者(轻度纤维化9例,中度14例,重度13例)皮损中Ⅰ、Ⅲ、Ⅴ型胶原α1链〔α1(Ⅰ)、α1(Ⅲ)、α1(Ⅴ)〕的表达(以平均光密度值为半定量检测指标),并以6例正常皮肤作为对照。结果3种胶原在SSc皮损真皮呈弥漫性分布,在网状层可见深染的α1(Ⅰ)和α1(Ⅲ)团块或条带,α1(Ⅴ)分布则较均匀,在小血管和皮肤附属器周围较多;从对照到SSc轻度、中度、重度纤维化组,α1(Ⅰ)、α1(Ⅲ)和α1(Ⅴ)含量在真皮、小血管和附属器及其周围组织均呈逐渐升高趋势(P均<0.05);在SSc皮损真皮、小血管和附属器及其周围组织中,α1(Ⅴ)在早期即明显升高(80.07±1.80 vs 73.56±2.48,94.23±1.53 vs 85.73±1.70,94.06±1.64 vs 84.65±1.65,P均<0.01),持续整个纤维化过程且增幅较大,α1(Ⅰ)和α1(Ⅲ)升高则较迟,在中、后期显著升高。结论α1(Ⅴ)先于α1(Ⅰ)、α1(Ⅲ)在SSc早期即开始大量沉积,并持续整个纤维化过程;其含量改变始于真皮小血管和附属器及其周围组织,可能参与SSc血管炎的发生。
基金This work was supported by the State Key Laboratory of Structural Chemistry (030065) the Chinese Academy of Sciences the NNSFC (20073048) and the NSF of Fujian province (2002F015)
文摘The crystal structure of the title compound, [enH2][Fe{MoⅤ6O12(OH)3(HPO4)- (H2PO4)3}2]6en6H2O (en = H2NCH2CH2NH2), hydrothermally synthesized from a mixture of Na2MoO42H2O, Fe2(SO4)3, H3PO4, H2N(CH2)2NH2 and water, has been determined by single- crystal X-ray diffraction. The crystal is of triclinic, space group P?with a = 11.9014(1), b = 13.4246(2), c = 13.8719(2) , a = 87.465(1), b = 69.981(1), g = 64.960(1)? V = 1873.46(4) 3, Z = 1, Mr = 2997.89, F(000) = 1466, m = 2.427 mm-1 and Dc = 2.657 g/cm3. The final R = 0.0404 for 5570 observed reflections (I > 2s(I)). The structural analysis reveals that each cluster anion contains two coplanar {Mo6} rings of six edge-sharing Mo(O5OH) octahedra, and the two {Mo6} rings are linked together through one octahedral FeⅡ ion to generate a sandwich-type cluster with rigorous () symmetry. Moreover, these clusters are further linked into a three-dimensional frame- work by hydrogen bonds.