GEMIN5 is a predominantly cytoplasmic multifunctional protein, known to be involved in recognizing snRNAs through its WD40 repeats domain placed at the N-terminus. A dimerization domain in the middle region acts as a ...GEMIN5 is a predominantly cytoplasmic multifunctional protein, known to be involved in recognizing snRNAs through its WD40 repeats domain placed at the N-terminus. A dimerization domain in the middle region acts as a hub for protein–protein interaction, while a non-canonical RNA-binding site is placed towards the C-terminus. The singular organization of structural domains present in GEMIN5 enables this protein to perform multiple functions through its ability to interact with distinct partners, both RNAs and proteins. This protein exerts a different role in translation regulation depending on its physiological state, such that while GEMIN5 down-regulates global RNA translation, the C-terminal half of the protein promotes translation of its mRNA. Additionally, GEMIN5 is responsible for the preferential partitioning of mRNAs into polysomes. Besides selective translation, GEMIN5 forms part of distinct ribonucleoprotein complexes, reflecting the dynamic organization of macromolecular complexes in response to internal and external signals. In accordance with its contribution to fundamental cellular processes, recent reports described clinical loss of function mutants suggesting that GEMIN5 deficiency is detrimental to cell growth and survival. Remarkably, patients carrying GEMIN5 biallelic variants suffer from neurodevelopmental delay, hypotonia, and cerebellar ataxia. Molecular analyses of individual variants, which are defective in protein dimerization, display decreased levels of ribosome association, reinforcing the involvement of the protein in translation regulation. Importantly, the number of clinical variants and the phenotypic spectrum associated with GEMIN5 disorders is increasing as the knowledge of the protein functions and the pathways linked to its activity augments. Here we discuss relevant advances concerning the functional and structural features of GEMIN5 and its separate domains in RNA-binding, protein interactome, and translation regulation, and how these data can help to understand the involvement of protein malfunction in clinical variants found in patients developing neurodevelopmental disorders.展开更多
It is shown that in the quantum structural approach to high-Tc superconductivity, the wave function in terms of the alternate molecular bonding geminals possesses off-diagonal long-range order (ODLRO).
The recent identification of a neurodevelopmental disorder with cerebellar atrophy and motor dysfunction(NEDCAM)has resulted in an increased interest in GEMIN5,a multifunction RNA-binding protein.As the largest member...The recent identification of a neurodevelopmental disorder with cerebellar atrophy and motor dysfunction(NEDCAM)has resulted in an increased interest in GEMIN5,a multifunction RNA-binding protein.As the largest member of the survival motor neuron complex,GEMIN5 plays a key role in the biogenesis of small nuclear ribonucleoproteins while also exhibiting translational regulatory functions as an independent protein.Although many questions remain regarding both the pathogenesis and pathophysiology of this new disorder,considerable progress has been made in the brief time since its discovery.In this review,we examine GEMIN5 within the context of NEDCAM,focusing on the structure,function,and expression of the protein specifically in regard to the disorder itself.Additionally,we explore the current animal models of NEDCAM,as well as potential molecular pathways for treatment and future directions of study.This review provides a comprehensive overview of recent advances in our understanding of this unique member of the survival motor neuron complex.展开更多
The ring-opening polymerization of heterocyclic monomers and the reversed ring-closing depolymerization of corresponding polymers with neutral thermodynamics are broadly explored to establish a circular economy of nex...The ring-opening polymerization of heterocyclic monomers and the reversed ring-closing depolymerization of corresponding polymers with neutral thermodynamics are broadly explored to establish a circular economy of next-generation plastics.Polythioesters(PTEs),analogues of polyesters,are emerging materials for this purpose due to their high refractive index,high crystallinity,dynamic property and responsiveness.In this work,we synthesize and polymerize a series of D-penicillamine-derivedβ-thiolactones(NRPenTL)with varied side chain alkyl groups,and study the structure-property relationship of the resulting polymers.The obtained PTEs exhibit tunable glass transition temperature in a wide range of 130–50℃,and melting temperature of 90–105℃.In addition,copolymerizations of monomers with different side chains are effective in modulating material properties.The obtained homo and copolymers can be fully depolymerized to recycle monomers.This work provides a robust molecular platform and detailed structure-property relationship of PTEs with potential of achieving sustainable plastics.展开更多
We use the method of device simulation to study the losses and influences of geminate and bimolecular recombinations on the performances and properties of the bulk heterojunction organic solar cells. We find that a fr...We use the method of device simulation to study the losses and influences of geminate and bimolecular recombinations on the performances and properties of the bulk heterojunction organic solar cells. We find that a fraction of electrons（holes）in the device are collected by anode（cathode）. The direction of the corresponding current is opposite to the direction of photocurrent. And the current density increases with the bias increasing but decreases as bimolecular recombination（BR）or geminate recombination（GR） intensity increases. The maximum power, short circuit current, and fill factor display a stronger dependence on GR than on BR. While the influences of GR and BR on open circuit voltage are about the same.Our studies shed a new light on the loss mechanism and may provide a new way of improving the efficiency of bulk heterojunction organic solar cells.展开更多
Objective To investigate the combination of Epstein-Barr virus nuclear protein 3C (EBNA3C) with Gemin3 and its effect on Gemin3 expression. Methods Co-immunoprecipitation, GST pull-down and immunofluorescent assay wer...Objective To investigate the combination of Epstein-Barr virus nuclear protein 3C (EBNA3C) with Gemin3 and its effect on Gemin3 expression. Methods Co-immunoprecipitation, GST pull-down and immunofluorescent assay were used to determine the combination of EBNA3C and Gemin3 and their combining domain. Stable EBNA3C knockdown cell lines were made by lentivirus-delivered small hairpin RNA and then puromycin selection. Western blot was used to check the effect of EBNA3C on Gemin3 expression. Results EBNA3C and Gemin3 combined with each other in vivo and in vitro through their C-terminals. EBNA3C up-regulated Gemin3 gene expression. Conclusion EBNA3C forms complex with Gemin3 and up-regulates its expression.展开更多
Developmental anomalies can occur as a result of conjoining or twinning defects. These include fusion, gemination and concrescence. Such anomalies occur more often in the deciduous than in the permanent dentition and ...Developmental anomalies can occur as a result of conjoining or twinning defects. These include fusion, gemination and concrescence. Such anomalies occur more often in the deciduous than in the permanent dentition and only a few cases involving molar and premolar teeth have been reported in the literature. Cases of bilateral double teeth are less frequent than unilateral and prevalence seems to be higher in the anterior region. The present article is a rare case report of simultaneous occurrence of double teeth in permanent dentition involving the mandibular premolar-molar teeth in a 45-year-old male patient. Clinical observation along with radiographic examination was used to arrive at a diagnosis.展开更多
The present paper explores the special behavior of geminate consonants in Moroccan Arabic vis-á-vis short consonants and consonant clusters.By way of comparison,it is shown that geminates exhibit properties that ...The present paper explores the special behavior of geminate consonants in Moroccan Arabic vis-á-vis short consonants and consonant clusters.By way of comparison,it is shown that geminates exhibit properties that are reminiscent of both unit structures and cluster structures.In particular,we reveal that geminates in MA demonstrate an inconsistent behavior in relation to the process of schwa epenthesis.In this context,we ask whether geminates get split up in MA,and when and how that happens.In order to characterize the patterning of geminates in MA,different phonological representations of geminates are examined against the variable behavior of geminates.On this basis,it is eventually suggested that geminates should be depicted as two root nodes that are underlyingly associated with a mora at the prosodic level.展开更多
The question "why are the variation trends of R-X bond dissociation energy different?" is answered. The R-X bond dissociation energy (BDE) may be influenced by three main factors: the C-X intrinsic bond ener...The question "why are the variation trends of R-X bond dissociation energy different?" is answered. The R-X bond dissociation energy (BDE) may be influenced by three main factors: the C-X intrinsic bond energy, the 1,3 geminal repulsion, and the intramolecular charge-induced dipole. In the presence of atom X, the variation trend of BDE in R-X (R= Me, Et, i-Pr, t-Bu) is dominated by two factors, the 1,3 geminal repulsion and the intramolecular charge-induced dipole. The former decreases the R-X BDE, and the latter either increases or decreases the R-X BDE. For the series of R-X with the R-C bond (such as R-Me, R-CH == CH2, R-C≡CH, and R-CN), the 1,3 geminal repulsion decreases the R-X BDE, and the variation trends of R-C BDE decrease from Me to t-Bu. As regards the series of R-X (such as R-H, R-BH2, and R-SiH2) in which the electronegativity of atom X is smaller than that of the carbon atom, the above two factors decrease the R-X BDE, and the variation trends of the R-X BDE decrease from Me to t-Bu. As to the series of R-X (such as R-F, R-OH, R-Cl, R-Br, R-I, and R-NH2) in which the electronegativity of atom X is larger than that of the carbon atom, the 1,3 geminal repulsion decreases the R-X BDE, while the intramolecular charge-induced dipole increases the R-X BDE. In this case, the variation trends of R-X BDE depend on the competition of the two factors. As a result, some of them (e. g., R-F, R-OH) increase from Me to t-Bu, some (e. g., R-I) decrease from Me to t-Bu, and some (e. g., R-Br) change very little.展开更多
A series of novel tricyclic O,N-heterocycles, [1,2,4]triazolo[3,2-d][1,5]benzoxazepin-2-thiones 7 were achieved via acid-induced ring closure of the geminal arylazo isothiocyanate compounds 5 which were derived from s...A series of novel tricyclic O,N-heterocycles, [1,2,4]triazolo[3,2-d][1,5]benzoxazepin-2-thiones 7 were achieved via acid-induced ring closure of the geminal arylazo isothiocyanate compounds 5 which were derived from substituted chroman-4-ones, followed by feasible ring expansion with simultaneous insertion of the nitrogen atom into the carbon skeleton. The X-ray crystal structure of 7d was also described.展开更多
基金partially supported by grants PID2020-115096RB-I00 and PID2023-148273NB-I00 from Ministerio de Ciencia y Universidad (MICIU/AEI)(to EMS)。
文摘GEMIN5 is a predominantly cytoplasmic multifunctional protein, known to be involved in recognizing snRNAs through its WD40 repeats domain placed at the N-terminus. A dimerization domain in the middle region acts as a hub for protein–protein interaction, while a non-canonical RNA-binding site is placed towards the C-terminus. The singular organization of structural domains present in GEMIN5 enables this protein to perform multiple functions through its ability to interact with distinct partners, both RNAs and proteins. This protein exerts a different role in translation regulation depending on its physiological state, such that while GEMIN5 down-regulates global RNA translation, the C-terminal half of the protein promotes translation of its mRNA. Additionally, GEMIN5 is responsible for the preferential partitioning of mRNAs into polysomes. Besides selective translation, GEMIN5 forms part of distinct ribonucleoprotein complexes, reflecting the dynamic organization of macromolecular complexes in response to internal and external signals. In accordance with its contribution to fundamental cellular processes, recent reports described clinical loss of function mutants suggesting that GEMIN5 deficiency is detrimental to cell growth and survival. Remarkably, patients carrying GEMIN5 biallelic variants suffer from neurodevelopmental delay, hypotonia, and cerebellar ataxia. Molecular analyses of individual variants, which are defective in protein dimerization, display decreased levels of ribosome association, reinforcing the involvement of the protein in translation regulation. Importantly, the number of clinical variants and the phenotypic spectrum associated with GEMIN5 disorders is increasing as the knowledge of the protein functions and the pathways linked to its activity augments. Here we discuss relevant advances concerning the functional and structural features of GEMIN5 and its separate domains in RNA-binding, protein interactome, and translation regulation, and how these data can help to understand the involvement of protein malfunction in clinical variants found in patients developing neurodevelopmental disorders.
基金Project (No. 29892168) supported by the National Natural Science Foundation of China.
文摘It is shown that in the quantum structural approach to high-Tc superconductivity, the wave function in terms of the alternate molecular bonding geminals possesses off-diagonal long-range order (ODLRO).
基金supported by the U.S.National Institutes of Health(NIH)National Institute of Neurological Disorders and Stroke(NINDS),No.R01 NS134215(to UBP).
文摘The recent identification of a neurodevelopmental disorder with cerebellar atrophy and motor dysfunction(NEDCAM)has resulted in an increased interest in GEMIN5,a multifunction RNA-binding protein.As the largest member of the survival motor neuron complex,GEMIN5 plays a key role in the biogenesis of small nuclear ribonucleoproteins while also exhibiting translational regulatory functions as an independent protein.Although many questions remain regarding both the pathogenesis and pathophysiology of this new disorder,considerable progress has been made in the brief time since its discovery.In this review,we examine GEMIN5 within the context of NEDCAM,focusing on the structure,function,and expression of the protein specifically in regard to the disorder itself.Additionally,we explore the current animal models of NEDCAM,as well as potential molecular pathways for treatment and future directions of study.This review provides a comprehensive overview of recent advances in our understanding of this unique member of the survival motor neuron complex.
基金supported by the National Science Fund for Distinguished Young Scholars(No.22125101).
文摘The ring-opening polymerization of heterocyclic monomers and the reversed ring-closing depolymerization of corresponding polymers with neutral thermodynamics are broadly explored to establish a circular economy of next-generation plastics.Polythioesters(PTEs),analogues of polyesters,are emerging materials for this purpose due to their high refractive index,high crystallinity,dynamic property and responsiveness.In this work,we synthesize and polymerize a series of D-penicillamine-derivedβ-thiolactones(NRPenTL)with varied side chain alkyl groups,and study the structure-property relationship of the resulting polymers.The obtained PTEs exhibit tunable glass transition temperature in a wide range of 130–50℃,and melting temperature of 90–105℃.In addition,copolymerizations of monomers with different side chains are effective in modulating material properties.The obtained homo and copolymers can be fully depolymerized to recycle monomers.This work provides a robust molecular platform and detailed structure-property relationship of PTEs with potential of achieving sustainable plastics.
基金supported by grants from the National Natural Science Foundation of China(No.81171649)National Natural Science Foundation of Liaoning Province(No.201102267)
基金Project supported by the Natural Science Foundation of Hebei Province,China(Grant No.A2012203016)the Science Fund from the Education Department of Hebei Province,China(Grant Nos.QN20131103 and Z2009114)+1 种基金the Doctor Foundation of Yanshan University,China(Grant No.B580)the Young Teachers’Research Project of Yanshan University,China(Grant No.13LGB028)
文摘We use the method of device simulation to study the losses and influences of geminate and bimolecular recombinations on the performances and properties of the bulk heterojunction organic solar cells. We find that a fraction of electrons（holes）in the device are collected by anode（cathode）. The direction of the corresponding current is opposite to the direction of photocurrent. And the current density increases with the bias increasing but decreases as bimolecular recombination（BR）or geminate recombination（GR） intensity increases. The maximum power, short circuit current, and fill factor display a stronger dependence on GR than on BR. While the influences of GR and BR on open circuit voltage are about the same.Our studies shed a new light on the loss mechanism and may provide a new way of improving the efficiency of bulk heterojunction organic solar cells.
文摘Objective To investigate the combination of Epstein-Barr virus nuclear protein 3C (EBNA3C) with Gemin3 and its effect on Gemin3 expression. Methods Co-immunoprecipitation, GST pull-down and immunofluorescent assay were used to determine the combination of EBNA3C and Gemin3 and their combining domain. Stable EBNA3C knockdown cell lines were made by lentivirus-delivered small hairpin RNA and then puromycin selection. Western blot was used to check the effect of EBNA3C on Gemin3 expression. Results EBNA3C and Gemin3 combined with each other in vivo and in vitro through their C-terminals. EBNA3C up-regulated Gemin3 gene expression. Conclusion EBNA3C forms complex with Gemin3 and up-regulates its expression.
文摘Developmental anomalies can occur as a result of conjoining or twinning defects. These include fusion, gemination and concrescence. Such anomalies occur more often in the deciduous than in the permanent dentition and only a few cases involving molar and premolar teeth have been reported in the literature. Cases of bilateral double teeth are less frequent than unilateral and prevalence seems to be higher in the anterior region. The present article is a rare case report of simultaneous occurrence of double teeth in permanent dentition involving the mandibular premolar-molar teeth in a 45-year-old male patient. Clinical observation along with radiographic examination was used to arrive at a diagnosis.
文摘The present paper explores the special behavior of geminate consonants in Moroccan Arabic vis-á-vis short consonants and consonant clusters.By way of comparison,it is shown that geminates exhibit properties that are reminiscent of both unit structures and cluster structures.In particular,we reveal that geminates in MA demonstrate an inconsistent behavior in relation to the process of schwa epenthesis.In this context,we ask whether geminates get split up in MA,and when and how that happens.In order to characterize the patterning of geminates in MA,different phonological representations of geminates are examined against the variable behavior of geminates.On this basis,it is eventually suggested that geminates should be depicted as two root nodes that are underlyingly associated with a mora at the prosodic level.
基金Supported by the National Natural Science Foundation of China (Grant No. 20772028)the Natural Science Foundation of Hunan Province (Grant No. 06JJ2002)Key Laboratory of Theoretical Chemistry and Molecular Simulation of Ministry of Education, Hunan University of Science and Technology
文摘The question "why are the variation trends of R-X bond dissociation energy different?" is answered. The R-X bond dissociation energy (BDE) may be influenced by three main factors: the C-X intrinsic bond energy, the 1,3 geminal repulsion, and the intramolecular charge-induced dipole. In the presence of atom X, the variation trend of BDE in R-X (R= Me, Et, i-Pr, t-Bu) is dominated by two factors, the 1,3 geminal repulsion and the intramolecular charge-induced dipole. The former decreases the R-X BDE, and the latter either increases or decreases the R-X BDE. For the series of R-X with the R-C bond (such as R-Me, R-CH == CH2, R-C≡CH, and R-CN), the 1,3 geminal repulsion decreases the R-X BDE, and the variation trends of R-C BDE decrease from Me to t-Bu. As regards the series of R-X (such as R-H, R-BH2, and R-SiH2) in which the electronegativity of atom X is smaller than that of the carbon atom, the above two factors decrease the R-X BDE, and the variation trends of the R-X BDE decrease from Me to t-Bu. As to the series of R-X (such as R-F, R-OH, R-Cl, R-Br, R-I, and R-NH2) in which the electronegativity of atom X is larger than that of the carbon atom, the 1,3 geminal repulsion decreases the R-X BDE, while the intramolecular charge-induced dipole increases the R-X BDE. In this case, the variation trends of R-X BDE depend on the competition of the two factors. As a result, some of them (e. g., R-F, R-OH) increase from Me to t-Bu, some (e. g., R-I) decrease from Me to t-Bu, and some (e. g., R-Br) change very little.
基金Project supported by the National Natural Science Foundation of China (No. 20372015) and the Key Organic Synthesis Laboratory of Jiangsu Province (No. KJS01016).
文摘A series of novel tricyclic O,N-heterocycles, [1,2,4]triazolo[3,2-d][1,5]benzoxazepin-2-thiones 7 were achieved via acid-induced ring closure of the geminal arylazo isothiocyanate compounds 5 which were derived from substituted chroman-4-ones, followed by feasible ring expansion with simultaneous insertion of the nitrogen atom into the carbon skeleton. The X-ray crystal structure of 7d was also described.
