Spiropolymers have gained a great deal of interest from both academic and industrial fields by virtue of their unique geometric structures and physical properties.Herein,we prepared a series of spirocopolymers through...Spiropolymers have gained a great deal of interest from both academic and industrial fields by virtue of their unique geometric structures and physical properties.Herein,we prepared a series of spirocopolymers through the catalyst-free four-component spiropolymerization of diisocyanides,activated alkynes,and two different kinds of monomers with reactive carbonyl groups.It is found that the polymerization reactivity of monomers,feeding modes,and feed ratios play significant roles in spirocopolymerization.Monomers with high reactivity and feeding reactive monomers first contribute to improving the molecular weights and yields of the polymers.The constructed copolymers have two different kinds of spiro structures,which is confirmed by the nuclear magnetic resonance.In addition,the spirocopolymers display the unique cluster-triggered emission and aggregation-induced emission properties,and their emission properties can be well-modulated by altering the ratio of comonomers.It is highly anticipated that this line of research will enrich the methodology of multi-component spiropolymerization,and provide a new insight into developing spiropolymers with various spiro structures and tunable properties.展开更多
Objectives:Hearing loss is a worldwide disease.In 50%of the patients,hearing loss is caused by genetic problems associated with GJB2,MTRNR1,SLC26A4,and other genes.Considering the recent development and cost reduction...Objectives:Hearing loss is a worldwide disease.In 50%of the patients,hearing loss is caused by genetic problems associated with GJB2,MTRNR1,SLC26A4,and other genes.Considering the recent development and cost reduction of whole-exome sequencing,it is possible to filter out the normal genes and find which among the more novel genes contributed to the loss of hearing.Methods:After prescreening all individuals for GJB2,MTRNR1 and SLC26A4 mutations,whole-exome sequencing was performed in the proband,and the pathogenic variant was confirmed via Sanger sequencing.Results:The compound-heterozygous variant namely c.8076G>C:p.E2692D and c.6362T>C:p.V2121A in OTOG was identified as a candidate gene of a consanguineous Kazakh family.Conclusion:This is the first reported case of severe deafness caused by an OTOG compound-heterozygous variant in the world and the first case of deafness caused by an OTOG variant in China.This discovery identified the important contribution of OTOG toward deafness and expanded the spectrum of variants responsible for human hearing loss.展开更多
基金supported by the National Natural Science Foundation of China(Nos.21875019,22175023,21975020 and 21975021)the National Key Research and Development Program of China(No.2018YFA0901800)+1 种基金College Students’Innovative Entrepreneurial Training Plan Program(No.BIT2022LH180)Beijing Institute of Technology Research Fund Program for Young Scholars.
文摘Spiropolymers have gained a great deal of interest from both academic and industrial fields by virtue of their unique geometric structures and physical properties.Herein,we prepared a series of spirocopolymers through the catalyst-free four-component spiropolymerization of diisocyanides,activated alkynes,and two different kinds of monomers with reactive carbonyl groups.It is found that the polymerization reactivity of monomers,feeding modes,and feed ratios play significant roles in spirocopolymerization.Monomers with high reactivity and feeding reactive monomers first contribute to improving the molecular weights and yields of the polymers.The constructed copolymers have two different kinds of spiro structures,which is confirmed by the nuclear magnetic resonance.In addition,the spirocopolymers display the unique cluster-triggered emission and aggregation-induced emission properties,and their emission properties can be well-modulated by altering the ratio of comonomers.It is highly anticipated that this line of research will enrich the methodology of multi-component spiropolymerization,and provide a new insight into developing spiropolymers with various spiro structures and tunable properties.
基金supported by the National Natural Science Foundation of China(No.81560166).
文摘Objectives:Hearing loss is a worldwide disease.In 50%of the patients,hearing loss is caused by genetic problems associated with GJB2,MTRNR1,SLC26A4,and other genes.Considering the recent development and cost reduction of whole-exome sequencing,it is possible to filter out the normal genes and find which among the more novel genes contributed to the loss of hearing.Methods:After prescreening all individuals for GJB2,MTRNR1 and SLC26A4 mutations,whole-exome sequencing was performed in the proband,and the pathogenic variant was confirmed via Sanger sequencing.Results:The compound-heterozygous variant namely c.8076G>C:p.E2692D and c.6362T>C:p.V2121A in OTOG was identified as a candidate gene of a consanguineous Kazakh family.Conclusion:This is the first reported case of severe deafness caused by an OTOG compound-heterozygous variant in the world and the first case of deafness caused by an OTOG variant in China.This discovery identified the important contribution of OTOG toward deafness and expanded the spectrum of variants responsible for human hearing loss.