The complex dimethylgallium- [4-nitro-2- (N-benzo- 15-crown-5 ) - 1 -phenoxide ] (C23H31 N2O7Ga) has been synthesized by the method reported. The crystal X-ray diffraction analysis shows that the crystal belongs to th...The complex dimethylgallium- [4-nitro-2- (N-benzo- 15-crown-5 ) - 1 -phenoxide ] (C23H31 N2O7Ga) has been synthesized by the method reported. The crystal X-ray diffraction analysis shows that the crystal belongs to the triclinic with space group P1, M = 517. 23 and the unit cell parameters: a = 1. 4501 (2), b = 1. 5090(2), c = 1. 3865 (6)nm, a = 105. 58(1 ), β = 117. 26(2), γ = 66. 812(8)°, V= 2. 464546nm3, Z =4, Dc = 1. 39g· cm-3, u = 11. 6cm-1, F(000) = 1080, R = 0. 051, Rw = 0. 064. The coordination number of Ga is four and the gallium atom is located in a distorted tetrahedron structure.展开更多
The title compound [ClCH2(C7H6N2)]NO3 has been prepared and characterized by elemental analysis and X-ray studies. It crystallizes in the monoclinic system, space group P21/n with a = 7.4189(3), b =15.3064(6), c = 9.2...The title compound [ClCH2(C7H6N2)]NO3 has been prepared and characterized by elemental analysis and X-ray studies. It crystallizes in the monoclinic system, space group P21/n with a = 7.4189(3), b =15.3064(6), c = 9.2657(3) ? b = 102.449(2)? C8H8ClN3O3, Mr = 229.62, V = 1027.44(7) 3, Z = 4, Dc = 1.484 g/cm3, F(000) = 472, = 0.363 mm-1, R = 0.0671 and wR = 0.1546. The crystal structure consists of discrete 2-chloromethyl-1H-benzimidazole cations and NO3- anions. The benzimidazole ring with the conjunction carbon atom C(1) is fairly planar, with the deviation from the least plane through the ring atoms is smaller than 0.010(3) ? The analytical results of crystal structure show that three different non-covalent interactions in the compound, NH…O intermolecular hydrogen bonds, CH…O interaction and p-p stacking interaction, play an important role in the crystal packing.展开更多
Benign familial chronic pemphigus (Hailey-Hailey disease,HHD) is a rare hereditary condition characterized by development of blisters at sites of friction and in the intertriginous areas. Mutations in the ATP2C1 gene,...Benign familial chronic pemphigus (Hailey-Hailey disease,HHD) is a rare hereditary condition characterized by development of blisters at sites of friction and in the intertriginous areas. Mutations in the ATP2C1 gene, which encodes the human secretory pathway calcium ATPase 1 (hSPCA1), have been identified as possible causative mutations. Studying Hungarian patients with HHD, we found two novel, distinct, heterozygous mutations. In a 65-year-old man with a 41-year history of severe recurrent symptoms, a single nucleotide insertion,1085insA, was detected. In a patient whose symptoms were induced by environmental contact allergens, we found a nonsense mutation, Q506X, in exon 17. Our study further illustrates the diversity of mutational events in the pathogenesis of HHD.展开更多
Hailey-Hailey disease(HHD;MIM 16960)is a rare autosomal dominant hereditary disorder characterized by recurrent eruption of vesicles and bullae,predominantly involving the body folds.It is caused by heterozygous mutat...Hailey-Hailey disease(HHD;MIM 16960)is a rare autosomal dominant hereditary disorder characterized by recurrent eruption of vesicles and bullae,predominantly involving the body folds.It is caused by heterozygous mutations in the ATP2C1 gene,encoding the human secretory pathway Ca2+/Mn-ATPase protein 1(hSPCA1).When we studied Chinese patients with HHD,we found two different heterozygous mutations,Q506X and G353V,the former previously reported ina Hungarian patient,and the latter being a novel mutation.In a 38-year-old patient from a four-generation pedigree with a3-year history of severe recurrent blisters,we identified a C →T transition at nucleotide 1696,c(1696C →T),in exon 17 of ATP2C1,resulting in a nonsenes mutation,Gln506X,which resultedin a premature termination codon.In the second patient,who represented a occurrence of sporadic Hailey-Hailey disease,a G →T transversion of nucleotide,c(G1238T),in exon13 of ATP2C1 was detected,which resulted in a Gly353 →Val amino acid substitution(G353V).Our molecular findings further demonstrate that the mutational events in the human ATP2C1 gene encoding the hSPCA1 pump play an important role in the pathogenesis of HHD.展开更多
文摘The complex dimethylgallium- [4-nitro-2- (N-benzo- 15-crown-5 ) - 1 -phenoxide ] (C23H31 N2O7Ga) has been synthesized by the method reported. The crystal X-ray diffraction analysis shows that the crystal belongs to the triclinic with space group P1, M = 517. 23 and the unit cell parameters: a = 1. 4501 (2), b = 1. 5090(2), c = 1. 3865 (6)nm, a = 105. 58(1 ), β = 117. 26(2), γ = 66. 812(8)°, V= 2. 464546nm3, Z =4, Dc = 1. 39g· cm-3, u = 11. 6cm-1, F(000) = 1080, R = 0. 051, Rw = 0. 064. The coordination number of Ga is four and the gallium atom is located in a distorted tetrahedron structure.
基金the Natural Science Foundation of Shandong province (No. Y2002B06)the Outstanding Adult-Young Scientific Research Encouraging Foundation of Shandong province (No. O1BS18)
文摘The title compound [ClCH2(C7H6N2)]NO3 has been prepared and characterized by elemental analysis and X-ray studies. It crystallizes in the monoclinic system, space group P21/n with a = 7.4189(3), b =15.3064(6), c = 9.2657(3) ? b = 102.449(2)? C8H8ClN3O3, Mr = 229.62, V = 1027.44(7) 3, Z = 4, Dc = 1.484 g/cm3, F(000) = 472, = 0.363 mm-1, R = 0.0671 and wR = 0.1546. The crystal structure consists of discrete 2-chloromethyl-1H-benzimidazole cations and NO3- anions. The benzimidazole ring with the conjunction carbon atom C(1) is fairly planar, with the deviation from the least plane through the ring atoms is smaller than 0.010(3) ? The analytical results of crystal structure show that three different non-covalent interactions in the compound, NH…O intermolecular hydrogen bonds, CH…O interaction and p-p stacking interaction, play an important role in the crystal packing.
文摘Benign familial chronic pemphigus (Hailey-Hailey disease,HHD) is a rare hereditary condition characterized by development of blisters at sites of friction and in the intertriginous areas. Mutations in the ATP2C1 gene, which encodes the human secretory pathway calcium ATPase 1 (hSPCA1), have been identified as possible causative mutations. Studying Hungarian patients with HHD, we found two novel, distinct, heterozygous mutations. In a 65-year-old man with a 41-year history of severe recurrent symptoms, a single nucleotide insertion,1085insA, was detected. In a patient whose symptoms were induced by environmental contact allergens, we found a nonsense mutation, Q506X, in exon 17. Our study further illustrates the diversity of mutational events in the pathogenesis of HHD.
文摘Hailey-Hailey disease(HHD;MIM 16960)is a rare autosomal dominant hereditary disorder characterized by recurrent eruption of vesicles and bullae,predominantly involving the body folds.It is caused by heterozygous mutations in the ATP2C1 gene,encoding the human secretory pathway Ca2+/Mn-ATPase protein 1(hSPCA1).When we studied Chinese patients with HHD,we found two different heterozygous mutations,Q506X and G353V,the former previously reported ina Hungarian patient,and the latter being a novel mutation.In a 38-year-old patient from a four-generation pedigree with a3-year history of severe recurrent blisters,we identified a C →T transition at nucleotide 1696,c(1696C →T),in exon 17 of ATP2C1,resulting in a nonsenes mutation,Gln506X,which resultedin a premature termination codon.In the second patient,who represented a occurrence of sporadic Hailey-Hailey disease,a G →T transversion of nucleotide,c(G1238T),in exon13 of ATP2C1 was detected,which resulted in a Gly353 →Val amino acid substitution(G353V).Our molecular findings further demonstrate that the mutational events in the human ATP2C1 gene encoding the hSPCA1 pump play an important role in the pathogenesis of HHD.
