Based on the data of 13 eco-environmental indicators of 14 prefecture level cities in Hunan Province from 2014 to 2018,this paper quantitatively evaluated the eco-environmental competitiveness and its advantages and d...Based on the data of 13 eco-environmental indicators of 14 prefecture level cities in Hunan Province from 2014 to 2018,this paper quantitatively evaluated the eco-environmental competitiveness and its advantages and disadvantages by principal component analysis(PCA).The results showed that:(1)the overall ecoenvironmental competitiveness of Hunan Province was relatively high,whereas the competitiveness level of each city and prefecture was quite different.The comprehensive score of eco-environmental competitiveness of Changsha City was 2.96,ranking the first,and Zhangjiajie city ranking the last with a score of-1.60,which indicated that there was an obvious difference in the level of eco-environmental competitiveness among different regions.(2)The overall eco-environmental competitiveness of cities and prefectures in Western Hunan Province was weak.Among the 14 prefectures and cities in Hunan Province,the comprehensive scores of eco-environmental competitiveness of 6 prefectures and cities were negative,whereas the scores of Zhangjiajie,Xiangxi and Huaihua in Western Hunan were all lower than the others.In the face of increasingly serious environmental problems,cities and prefectures should adjust measures according to local conditions and put forward specific measures to enhance environmental competitiveness.In particular,cities and prefectures in Western Hunan should give full play to their advantages in ecological resources,take the path of green development,enhance the competitiveness of ecological environment,and provide support for local economic development.展开更多
Background ADULT syndrome （acro-dermato-ungual-lacrimal-tooth syndrome） is a rare ectodermal dysplasia disorder known as autosomal dominant inheritance. Recent studies have linked p63 gene mutation to the developmen...Background ADULT syndrome （acro-dermato-ungual-lacrimal-tooth syndrome） is a rare ectodermal dysplasia disorder known as autosomal dominant inheritance. Recent studies have linked p63 gene mutation to the development of this disease. However, the genetic characteristics of ADULT syndrome were still not well understood. Methods Mutation analysis of p63 gene in the first Chinese ADULT syndrome family was performed using direct DNA sequencing. Results The sequence analysis of exon 8 of p63 gene disclosed a heterozygous G〉A substitution at nucleotide 893 （R298Q） in the proband. In addition, a single nucleotide polymorphism （SNP） rs16864880 in the downstream flanking region （DFR） of p63 exon 8 was also identified in this family. The proband and the paternal side including her father exhibited the C/G genotype at this position. The C/G variant frequency in the paternal was significantly higher as compared with the maternal （6/10 vs 0/6, P=0.034）. Conclusions ADULT syndrome may be caused by the p63 gene mutation, and it might have closer genetic association with the paternal side in this family.展开更多
Acro-dermato-ungual-lacrimal-tooth syndrome （ADULT syndrome, OMIM 103285） is a rarely seen ectodermal dysplasia disorder first described by Propping and Zerres in 1993. ADULT syndrome is known as an autosomal domina...Acro-dermato-ungual-lacrimal-tooth syndrome （ADULT syndrome, OMIM 103285） is a rarely seen ectodermal dysplasia disorder first described by Propping and Zerres in 1993. ADULT syndrome is known as an autosomal dominant disorder. Only a family constellation and four independent cases were reported worldwide up to now. Here, we report a case of ADULT syndrome, which is the first case reported from China.展开更多
文摘Based on the data of 13 eco-environmental indicators of 14 prefecture level cities in Hunan Province from 2014 to 2018,this paper quantitatively evaluated the eco-environmental competitiveness and its advantages and disadvantages by principal component analysis(PCA).The results showed that:(1)the overall ecoenvironmental competitiveness of Hunan Province was relatively high,whereas the competitiveness level of each city and prefecture was quite different.The comprehensive score of eco-environmental competitiveness of Changsha City was 2.96,ranking the first,and Zhangjiajie city ranking the last with a score of-1.60,which indicated that there was an obvious difference in the level of eco-environmental competitiveness among different regions.(2)The overall eco-environmental competitiveness of cities and prefectures in Western Hunan Province was weak.Among the 14 prefectures and cities in Hunan Province,the comprehensive scores of eco-environmental competitiveness of 6 prefectures and cities were negative,whereas the scores of Zhangjiajie,Xiangxi and Huaihua in Western Hunan were all lower than the others.In the face of increasingly serious environmental problems,cities and prefectures should adjust measures according to local conditions and put forward specific measures to enhance environmental competitiveness.In particular,cities and prefectures in Western Hunan should give full play to their advantages in ecological resources,take the path of green development,enhance the competitiveness of ecological environment,and provide support for local economic development.
基金This work was supported by a grant from Guangdong Natural Science Foundation of China （No. 06022455）.
文摘Background ADULT syndrome （acro-dermato-ungual-lacrimal-tooth syndrome） is a rare ectodermal dysplasia disorder known as autosomal dominant inheritance. Recent studies have linked p63 gene mutation to the development of this disease. However, the genetic characteristics of ADULT syndrome were still not well understood. Methods Mutation analysis of p63 gene in the first Chinese ADULT syndrome family was performed using direct DNA sequencing. Results The sequence analysis of exon 8 of p63 gene disclosed a heterozygous G〉A substitution at nucleotide 893 （R298Q） in the proband. In addition, a single nucleotide polymorphism （SNP） rs16864880 in the downstream flanking region （DFR） of p63 exon 8 was also identified in this family. The proband and the paternal side including her father exhibited the C/G genotype at this position. The C/G variant frequency in the paternal was significantly higher as compared with the maternal （6/10 vs 0/6, P=0.034）. Conclusions ADULT syndrome may be caused by the p63 gene mutation, and it might have closer genetic association with the paternal side in this family.
基金This study was supported by a grant from Guangdong Natural Science Foundation in 2006(No.06022455).
文摘Acro-dermato-ungual-lacrimal-tooth syndrome （ADULT syndrome, OMIM 103285） is a rarely seen ectodermal dysplasia disorder first described by Propping and Zerres in 1993. ADULT syndrome is known as an autosomal dominant disorder. Only a family constellation and four independent cases were reported worldwide up to now. Here, we report a case of ADULT syndrome, which is the first case reported from China.
