Type II autosomal dominant osteopetrosis(ADO2), which is the most common form of osteopetrosis, is caused by heterozygous mutations in the chloride channel 7(CLCN7) gene. The osteopetrosis of ADO2 has been attributed ...Type II autosomal dominant osteopetrosis(ADO2), which is the most common form of osteopetrosis, is caused by heterozygous mutations in the chloride channel 7(CLCN7) gene. The osteopetrosis of ADO2 has been attributed to hypofunctional osteoclasts. The mechanism underlying the abnormality in osteoclast function remains largely unknown. This study was designed to investigate gene mutations and osteoclast function in a case that was clinically diagnosed as ADO2. Genomic DNA was extracted from blood samples of this patient, and the 25 exons of CLCN7 were amplified. Peripheral blood from the ADO2 subject and a healthy age- and sex-matched control was used to evaluate osteoclastogenesis, osteoclast morphology, and bone resorption. Analysis of DNA from the patient showed a germline heterozygous missense mutation,c.1856C>T(p.P619L), in exon 20 of CLCN7. A similar homozygous mutation at this site was previously reported in a patient with autosomal recessive osteopetrosis. When cultured, the peripheral blood mononuclear cells(PBMCs) from the ADO2 patient spontaneously differentiated into mature osteoclasts in vitro. The ADO2 patient’s PBMCs formed enhanced, but heterogeneous, osteoclasts in both the presence and absence of macrophage-colony stimulating factor, and nuclear factor-?B ligand. Bone resorption was reduced in the ADO2 patient’s osteoclasts, which exhibited aberrant morphology and abnormal distribution of integrin avβ3. Gene analysis found increased c-fos expression and reduced Rho A and integrin beta 3expression in ADO2 cells. In conclusion, our data suggest that enhanced, heterogeneous osteoclast induction may be an intrinsic characteristic of ADO2.展开更多
Chloride ion critical content was studied under soaking and cycle of dry and wet conditions, with three electrochemical nondestructive measuring techniques, i e, half-cell potential, A C impedance, and time potential....Chloride ion critical content was studied under soaking and cycle of dry and wet conditions, with three electrochemical nondestructive measuring techniques, i e, half-cell potential, A C impedance, and time potential. The experimental results show that chloride ion critical content is primarily determined by the water cement ratio, while for the same concrete mixture the chloride ion critical content in soaking conditions is larger than that in a cycle of dry and wet conditions.展开更多
A new structure for efficient and cost-effective L-band amplified spontaneous emission (ASE) generation was experimentally investigated and demonstrated using a C/L-band coupler, an optical mirror, and a fiber Bragg g...A new structure for efficient and cost-effective L-band amplified spontaneous emission (ASE) generation was experimentally investigated and demonstrated using a C/L-band coupler, an optical mirror, and a fiber Bragg grating (FBG). The proposed structure has about 1.5 dB increase of the power around 1570 nm compared with conventional one. Various structures for power enhancement were also considered.展开更多
基金supported by grants from the National Natural Science Foundation of China(Nos.81572639,81370969 and 81072190 to X Yu)the Ministry of Education of the People's Republic of China(No.20130181110066 to X Yu)the Chengdu Bureau of Science and Technology(No.2014-HM01-00382-SF to X Yu)
文摘Type II autosomal dominant osteopetrosis(ADO2), which is the most common form of osteopetrosis, is caused by heterozygous mutations in the chloride channel 7(CLCN7) gene. The osteopetrosis of ADO2 has been attributed to hypofunctional osteoclasts. The mechanism underlying the abnormality in osteoclast function remains largely unknown. This study was designed to investigate gene mutations and osteoclast function in a case that was clinically diagnosed as ADO2. Genomic DNA was extracted from blood samples of this patient, and the 25 exons of CLCN7 were amplified. Peripheral blood from the ADO2 subject and a healthy age- and sex-matched control was used to evaluate osteoclastogenesis, osteoclast morphology, and bone resorption. Analysis of DNA from the patient showed a germline heterozygous missense mutation,c.1856C>T(p.P619L), in exon 20 of CLCN7. A similar homozygous mutation at this site was previously reported in a patient with autosomal recessive osteopetrosis. When cultured, the peripheral blood mononuclear cells(PBMCs) from the ADO2 patient spontaneously differentiated into mature osteoclasts in vitro. The ADO2 patient’s PBMCs formed enhanced, but heterogeneous, osteoclasts in both the presence and absence of macrophage-colony stimulating factor, and nuclear factor-?B ligand. Bone resorption was reduced in the ADO2 patient’s osteoclasts, which exhibited aberrant morphology and abnormal distribution of integrin avβ3. Gene analysis found increased c-fos expression and reduced Rho A and integrin beta 3expression in ADO2 cells. In conclusion, our data suggest that enhanced, heterogeneous osteoclast induction may be an intrinsic characteristic of ADO2.
文摘Chloride ion critical content was studied under soaking and cycle of dry and wet conditions, with three electrochemical nondestructive measuring techniques, i e, half-cell potential, A C impedance, and time potential. The experimental results show that chloride ion critical content is primarily determined by the water cement ratio, while for the same concrete mixture the chloride ion critical content in soaking conditions is larger than that in a cycle of dry and wet conditions.
文摘A new structure for efficient and cost-effective L-band amplified spontaneous emission (ASE) generation was experimentally investigated and demonstrated using a C/L-band coupler, an optical mirror, and a fiber Bragg grating (FBG). The proposed structure has about 1.5 dB increase of the power around 1570 nm compared with conventional one. Various structures for power enhancement were also considered.
