Retinitis pigmentosa is a leading cause of blindness and a progressive retinal disorder,affecting millions of people worldwide.This disease is characterized by photoreceptor degeneration,eventually leading to complete...Retinitis pigmentosa is a leading cause of blindness and a progressive retinal disorder,affecting millions of people worldwide.This disease is characterized by photoreceptor degeneration,eventually leading to complete blindness.Autosomal dominant(adRP)has been associated with mutations in at least four ubiquitously expressed genes encoding pre-mRNA splicing factors—Prp3,Prp8,Prp31 and PAP1.Biological function of adRPassociated splicing factor genes and molecular mechanisms by which mutations in these genes cause cell-type specific photoreceptor degeneration in humans remain to be elucidated.To investigate the in vivo function of these adRP-associated splicing factor genes,we examined Drosophila in which expression of fly Prp31 homolog was down-regulated.Sequence analyses show that CG6876 is the likely candidate of Drosophila melanogaster Prp31 homolog(DmPrp31).Predicted peptide sequence for CG6876 shows 57%similarity to the Homo sapiens Prp31 protein(HsPrp31).Reduction of the endogenous Prp31 by RNAi-mediated knockdown speci-fically in the eye leads to reduction of eye size or complete absence of eyes with remarkable features of photoreceptor degeneration and recapitulates the bimodal expressivity of human Prp31 mutations in adRP patients.Such transgenic DmPrp31RNAi flies provide a useful tool for identifying genetic modifiers or interacting genes for Prp31.Expression of the human Prp31 in these animals leads to a partial rescue of the eye phenotype.Our results indicate that the Drosophila CG6876 is the fly ortholog of mammalian Prp31 gene.展开更多
It has been formerly pointed out that the global average amplitude of the semidiurnal barometric component S2（P） at the ground is about two times that of the diurnal barometric component, S1（P）. Accordingly many m...It has been formerly pointed out that the global average amplitude of the semidiurnal barometric component S2（P） at the ground is about two times that of the diurnal barometric component, S1（P）. Accordingly many meteorologists have researched the cause why the solar semidiurnal barometric oscillation is magnified and the solar diurnal barometric oscillation is suppressed at the ground.展开更多
基金We acknowledge Ms.Tiffany Jean and members of the Wu lab for their help in preparation of the manuscript.We thank NIH(GM070967 and EY014576 to JYW)and Searle Foundation(to JYW)for grant support.
文摘Retinitis pigmentosa is a leading cause of blindness and a progressive retinal disorder,affecting millions of people worldwide.This disease is characterized by photoreceptor degeneration,eventually leading to complete blindness.Autosomal dominant(adRP)has been associated with mutations in at least four ubiquitously expressed genes encoding pre-mRNA splicing factors—Prp3,Prp8,Prp31 and PAP1.Biological function of adRPassociated splicing factor genes and molecular mechanisms by which mutations in these genes cause cell-type specific photoreceptor degeneration in humans remain to be elucidated.To investigate the in vivo function of these adRP-associated splicing factor genes,we examined Drosophila in which expression of fly Prp31 homolog was down-regulated.Sequence analyses show that CG6876 is the likely candidate of Drosophila melanogaster Prp31 homolog(DmPrp31).Predicted peptide sequence for CG6876 shows 57%similarity to the Homo sapiens Prp31 protein(HsPrp31).Reduction of the endogenous Prp31 by RNAi-mediated knockdown speci-fically in the eye leads to reduction of eye size or complete absence of eyes with remarkable features of photoreceptor degeneration and recapitulates the bimodal expressivity of human Prp31 mutations in adRP patients.Such transgenic DmPrp31RNAi flies provide a useful tool for identifying genetic modifiers or interacting genes for Prp31.Expression of the human Prp31 in these animals leads to a partial rescue of the eye phenotype.Our results indicate that the Drosophila CG6876 is the fly ortholog of mammalian Prp31 gene.
文摘It has been formerly pointed out that the global average amplitude of the semidiurnal barometric component S2（P） at the ground is about two times that of the diurnal barometric component, S1（P）. Accordingly many meteorologists have researched the cause why the solar semidiurnal barometric oscillation is magnified and the solar diurnal barometric oscillation is suppressed at the ground.
