The clinical diagnosis of a patient with the phenotype of EhlersDanlos syndrome type VI was confirmed biochemically by the severely diminished level of lysyl hydroxylase (LH) activity in the patient’s skin fibroblast...The clinical diagnosis of a patient with the phenotype of EhlersDanlos syndrome type VI was confirmed biochemically by the severely diminished level of lysyl hydroxylase (LH) activity in the patient’s skin fibroblasts. A novel homozygous mutation, a single base change of T1360 →G in exon 13 of the LH1 gene, predicted to result in W446G, was identified in the patient’s full-length cDNA. This was confirmed in genomic DNA from both the patient and her parents, who were heterozygous for the mutation. This mutation was introduced into an LH1-pAcGP67 baculoviral construct and expressed, in parallel with normal LH1, in an insect cell system. The loss of LH activity in the mutated recombinant construct confirmed the pathogenicity of this mutation. Although not in the major catalytic site, this mutation occurs in a highly conserved region of the LH1 gene and may contribute to loss of activity by interfering with normal folding of the enzyme.展开更多
Red-crowned Crane (Grus japonensis), a kind of endangered large-scale wader, is one of valuable and rare birds in the world. It has been indexed in China Red Data Book of Endangered Animals (Aves), China Data Book of ...Red-crowned Crane (Grus japonensis), a kind of endangered large-scale wader, is one of valuable and rare birds in the world. It has been indexed in China Red Data Book of Endangered Animals (Aves), China Data Book of Major Protection Wild Animals, World Red Data Book of Birds, State Trade Protocol of Endangered Wild Plants and Animals Species. The paper studies the amount, distribution, habit, growth and habitat of Red-crowned Crane in China in detail, systematically analyses the ecological environment feature of Red-crowned Crane breeding area, including the vegetation feature, the vertical distribution law of absolute humidity and temperature, some carnivorous food and herbivorous food for Red-crowned Crane. At present, the ecological environment of breeding area has been destroyed seriously by human activity. The area of marshland is reduced rapidly. A large area of reeds has been disappeared. The survival of Red-crowned Crane is threatened greatly. So it′s necessary to maintain the biodiversity of breeding area in order to protect Red-crowned Crane. .展开更多
The traditional urban form in historic Ningbo has undergone many radicaltransformations over the past two decades. The relatively homogeneous traditional fabric of the cityhas been damaged and remodeled following mode...The traditional urban form in historic Ningbo has undergone many radicaltransformations over the past two decades. The relatively homogeneous traditional fabric of the cityhas been damaged and remodeled following modernized urban models. This paperaims at synthesizingurban renewal and conservation through examining the revitalization of a particular traditionalurban area. The case of the West Yuehu area of Ningbo is selected to show the planning approachesand proposals, constraints and contradictions regarding revitalization under this social transition.The methodology used in the research is divided into two parts: literature review and field study.The theoretical part of the research regarding urban conservation in China is based on literaturereview. The field study is conducted by the authors and groups of students from SoutheastUniversity. The complete methodology attempts to illustrate the dimensions of urban renewal byexamining the processes, conflicts and results of revitalizationof historic urban areas, consideringthe present changing socio-economic context.展开更多
Both overgrazing and climate change contribute to grassland degradation in the alpine regions of China and negatively affect soil carbon and nitrogen pools. We quantified changes in soil organic carbon （SOC） and tot...Both overgrazing and climate change contribute to grassland degradation in the alpine regions of China and negatively affect soil carbon and nitrogen pools. We quantified changes in soil organic carbon （SOC） and total nitrogen （TN） in black soil beach （BSB）. We measured SOC and TN in severely degraded and non-degraded grasslands to calculate differences in carbon and nitrogen storage, and field survey results were extrapolated to the entire headwaters area of the Qinghai-Tibetan Plateau （36.3xlos krn~） to determine SOC and TN losses from these grasslands. We also evaluated changes in SOC and TN in severely degraded grasslands that were artificially re-vegetated five, seven and nine years ago. Totally 92.43 Tg C and 7.08 Tg N were lost from the BSB in the headwater area, which was approximately 50% of the original C and N soil pools. Re-vegetation of the degraded grasslands in the headwater area would result in a gain of 32.71 Tg C in the soil after five years, a loss of 5.5a Tg C after seven years and an increase of 44.15 Tg C after nine years. The TN increased by 53.09% and 59.98% after five and nine years, respectively, while it decreased by 4.92% after seven years of re-vegetation. The results indicate that C and N stocks followed a ＂V＂ shaped pattern with re- vegetation time. Understanding plant-soil interactions during succession of artificially planting grassland ecosystems is essential for developing scientifically sound management strategies for the effectively re-vegetated BSB.展开更多
Habitat loss and fragmentation are mainly associated with population decrease of endangered species and biodiversity loss. The habitat suitability maps of red-crowned crane （Grusjaponensis） in 1992, 1999 and 2006 we...Habitat loss and fragmentation are mainly associated with population decrease of endangered species and biodiversity loss. The habitat suitability maps of red-crowned crane （Grusjaponensis） in 1992, 1999 and 2006 were produced by using Ecological Niche Suitability Model （ENSM） in Yellow River Delta Nature Reserve （118°33′-119°20′E longitude, 37°35′-38°12′N latitude）, Shandong Province, China. Based on the habitat suitability maps, the causation and change law of habitat loss and fragmentation of red-crowned crane were analyzed by selecting a series of landscape pattern indices. Results showed that due to scarcities of fresh water sources, habitat suitability of red-crowned crane in 1999 was inferior to that in 1992 and 2006 no matter whether human disturbances existed or not. Besides, human disturbance activities, especially road disturbances, increased rapidly during the period of 1992-2006. This worsened the habitat loss and frag- mentation of red-crowned crane, and led to degrading habitat suitability of red-crowned crane in 2006, compared with that in 1992. In conclusion, fresh water sources and human disturbance activities are the two main factors that drive the habitat suitability change of red-crowned crane.展开更多
RIO(RED with IN and OUT) is the primary queue management mechanism proposed for assured forwarding in the DiffServ (Differentiated Service) framework. Although RIO can generally provide bandwidth guarantees, its queui...RIO(RED with IN and OUT) is the primary queue management mechanism proposed for assured forwarding in the DiffServ (Differentiated Service) framework. Although RIO can generally provide bandwidth guarantees, its queuing delay is sensitive to the traffic load. This paper presents a qualitative explanation for its origin. As a solution, an Adaptive RIO for Delay (ARIO-D) is proposed to provide guaranteed delay for multimedia traffic. Simulation results show that by trading loss for delay, ARIO-D can effectively improve the robustness of RIO under different and dynamic traffic, and provide stable and differentiated performance of queuing delay without any degradation in performance of throughput.展开更多
Common denominator and one of the goals in the ＂storage＂ of many, even different, commodities （agricultural, etc.） is their protection against invasion by biological pests; protection of objects of cultural herita...Common denominator and one of the goals in the ＂storage＂ of many, even different, commodities （agricultural, etc.） is their protection against invasion by biological pests; protection of objects of cultural heritage deposited in museums, galleries, archives and other institutions of a similar type is one of the specific case. The present article is an overview of basic （potential） biological pests of some ＂storage＂ commodities （with emphasis on the protection of objects of cultural heritage） as well as methods used for their detection. In the final section, a modular system of the ongoing assessment of the environmental characteristics of depositaries and exhibitions （including ＂biological＂） is briefly presented; its advantage is the possibility of on-line monitoring of the evaluated parameters （temperature, humidity, lighting, etc.） including the detection of presence of crawling and airborne insects.展开更多
Mobile pastoralist communities in Sahel countries have serious problems of access to social services. Classic strategies for social services were always based on a single sector, focused on settled populations, and ha...Mobile pastoralist communities in Sahel countries have serious problems of access to social services. Classic strategies for social services were always based on a single sector, focused on settled populations, and hardly reached mobile communities. This paper aims to show a cross-sector approach as a part of solutions to provide social services for mobile pastoralist communities and describes the ongoing scaling-up process. The method used is based on a transdisciplinary approach which involves target communities and authorities in all processes through workshops and site visits. Interventions adopted the concept of ＂one health＂ benefiting of an added value of closer cooperation between human and animal health. This approach was tested with joint interventions between human and animal health, which provided access to health services for unreached communities and saved resource when compared with single sector intervention. Such approaches could be extended to other social services within cross-sector interventions such as the delivery of basic education in nomadic areas. Such a strategy was validated by the communities and authorities and was further transformed into a national programme after a series of six national stakeholders＇ seminars. The cross-sector intervention will provide a minimum package of services （MPS） without compromising the structural services.展开更多
文摘The clinical diagnosis of a patient with the phenotype of EhlersDanlos syndrome type VI was confirmed biochemically by the severely diminished level of lysyl hydroxylase (LH) activity in the patient’s skin fibroblasts. A novel homozygous mutation, a single base change of T1360 →G in exon 13 of the LH1 gene, predicted to result in W446G, was identified in the patient’s full-length cDNA. This was confirmed in genomic DNA from both the patient and her parents, who were heterozygous for the mutation. This mutation was introduced into an LH1-pAcGP67 baculoviral construct and expressed, in parallel with normal LH1, in an insect cell system. The loss of LH activity in the mutated recombinant construct confirmed the pathogenicity of this mutation. Although not in the major catalytic site, this mutation occurs in a highly conserved region of the LH1 gene and may contribute to loss of activity by interfering with normal folding of the enzyme.
基金Under the auspices of Knowledge Innovation Project (No. KZCX2-302).
文摘Red-crowned Crane (Grus japonensis), a kind of endangered large-scale wader, is one of valuable and rare birds in the world. It has been indexed in China Red Data Book of Endangered Animals (Aves), China Data Book of Major Protection Wild Animals, World Red Data Book of Birds, State Trade Protocol of Endangered Wild Plants and Animals Species. The paper studies the amount, distribution, habit, growth and habitat of Red-crowned Crane in China in detail, systematically analyses the ecological environment feature of Red-crowned Crane breeding area, including the vegetation feature, the vertical distribution law of absolute humidity and temperature, some carnivorous food and herbivorous food for Red-crowned Crane. At present, the ecological environment of breeding area has been destroyed seriously by human activity. The area of marshland is reduced rapidly. A large area of reeds has been disappeared. The survival of Red-crowned Crane is threatened greatly. So it′s necessary to maintain the biodiversity of breeding area in order to protect Red-crowned Crane. .
文摘The traditional urban form in historic Ningbo has undergone many radicaltransformations over the past two decades. The relatively homogeneous traditional fabric of the cityhas been damaged and remodeled following modernized urban models. This paperaims at synthesizingurban renewal and conservation through examining the revitalization of a particular traditionalurban area. The case of the West Yuehu area of Ningbo is selected to show the planning approachesand proposals, constraints and contradictions regarding revitalization under this social transition.The methodology used in the research is divided into two parts: literature review and field study.The theoretical part of the research regarding urban conservation in China is based on literaturereview. The field study is conducted by the authors and groups of students from SoutheastUniversity. The complete methodology attempts to illustrate the dimensions of urban renewal byexamining the processes, conflicts and results of revitalizationof historic urban areas, consideringthe present changing socio-economic context.
基金financially supported by the grants from the Ministry of Science and Technology,China (Grant No. 2012BAC01B02)the Ministry of Environmental Protection,China (Grant No. 201209033)
文摘Both overgrazing and climate change contribute to grassland degradation in the alpine regions of China and negatively affect soil carbon and nitrogen pools. We quantified changes in soil organic carbon （SOC） and total nitrogen （TN） in black soil beach （BSB）. We measured SOC and TN in severely degraded and non-degraded grasslands to calculate differences in carbon and nitrogen storage, and field survey results were extrapolated to the entire headwaters area of the Qinghai-Tibetan Plateau （36.3xlos krn~） to determine SOC and TN losses from these grasslands. We also evaluated changes in SOC and TN in severely degraded grasslands that were artificially re-vegetated five, seven and nine years ago. Totally 92.43 Tg C and 7.08 Tg N were lost from the BSB in the headwater area, which was approximately 50% of the original C and N soil pools. Re-vegetation of the degraded grasslands in the headwater area would result in a gain of 32.71 Tg C in the soil after five years, a loss of 5.5a Tg C after seven years and an increase of 44.15 Tg C after nine years. The TN increased by 53.09% and 59.98% after five and nine years, respectively, while it decreased by 4.92% after seven years of re-vegetation. The results indicate that C and N stocks followed a ＂V＂ shaped pattern with re- vegetation time. Understanding plant-soil interactions during succession of artificially planting grassland ecosystems is essential for developing scientifically sound management strategies for the effectively re-vegetated BSB.
基金the research was supported by National Natural Science Foundation (No. 40771172)Knowledge innovation project of the Chinese Academy of Sciences (No. kzcx2-yw-308)
文摘Habitat loss and fragmentation are mainly associated with population decrease of endangered species and biodiversity loss. The habitat suitability maps of red-crowned crane （Grusjaponensis） in 1992, 1999 and 2006 were produced by using Ecological Niche Suitability Model （ENSM） in Yellow River Delta Nature Reserve （118°33′-119°20′E longitude, 37°35′-38°12′N latitude）, Shandong Province, China. Based on the habitat suitability maps, the causation and change law of habitat loss and fragmentation of red-crowned crane were analyzed by selecting a series of landscape pattern indices. Results showed that due to scarcities of fresh water sources, habitat suitability of red-crowned crane in 1999 was inferior to that in 1992 and 2006 no matter whether human disturbances existed or not. Besides, human disturbance activities, especially road disturbances, increased rapidly during the period of 1992-2006. This worsened the habitat loss and frag- mentation of red-crowned crane, and led to degrading habitat suitability of red-crowned crane in 2006, compared with that in 1992. In conclusion, fresh water sources and human disturbance activities are the two main factors that drive the habitat suitability change of red-crowned crane.
基金Supported by the National Natural Science Foundation of China(No.60202005) theFoundation for University Key Teacher by the Ministry of Education in P.R.Chinaalso supported in part by the Australian Research Council (No.LX0240468).
文摘RIO(RED with IN and OUT) is the primary queue management mechanism proposed for assured forwarding in the DiffServ (Differentiated Service) framework. Although RIO can generally provide bandwidth guarantees, its queuing delay is sensitive to the traffic load. This paper presents a qualitative explanation for its origin. As a solution, an Adaptive RIO for Delay (ARIO-D) is proposed to provide guaranteed delay for multimedia traffic. Simulation results show that by trading loss for delay, ARIO-D can effectively improve the robustness of RIO under different and dynamic traffic, and provide stable and differentiated performance of queuing delay without any degradation in performance of throughput.
文摘Common denominator and one of the goals in the ＂storage＂ of many, even different, commodities （agricultural, etc.） is their protection against invasion by biological pests; protection of objects of cultural heritage deposited in museums, galleries, archives and other institutions of a similar type is one of the specific case. The present article is an overview of basic （potential） biological pests of some ＂storage＂ commodities （with emphasis on the protection of objects of cultural heritage） as well as methods used for their detection. In the final section, a modular system of the ongoing assessment of the environmental characteristics of depositaries and exhibitions （including ＂biological＂） is briefly presented; its advantage is the possibility of on-line monitoring of the evaluated parameters （temperature, humidity, lighting, etc.） including the detection of presence of crawling and airborne insects.
文摘Mobile pastoralist communities in Sahel countries have serious problems of access to social services. Classic strategies for social services were always based on a single sector, focused on settled populations, and hardly reached mobile communities. This paper aims to show a cross-sector approach as a part of solutions to provide social services for mobile pastoralist communities and describes the ongoing scaling-up process. The method used is based on a transdisciplinary approach which involves target communities and authorities in all processes through workshops and site visits. Interventions adopted the concept of ＂one health＂ benefiting of an added value of closer cooperation between human and animal health. This approach was tested with joint interventions between human and animal health, which provided access to health services for unreached communities and saved resource when compared with single sector intervention. Such approaches could be extended to other social services within cross-sector interventions such as the delivery of basic education in nomadic areas. Such a strategy was validated by the communities and authorities and was further transformed into a national programme after a series of six national stakeholders＇ seminars. The cross-sector intervention will provide a minimum package of services （MPS） without compromising the structural services.
