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The splicing factor Prp31 is essential for photoreceptor development in Drosophila

Protein & Cell 2010年第3期1卷 267-274页

作者： payal ray Xiaoyan Luo Elizabeth J.Rao Amina Basha Elvin A.Woodruff III Jane Y.Wu Department of Neurology Northwestern University Feinberg Medical SchoolChicagoIL 60611USA Magnify Inc. 2316 Riverside DrNashvilleTN 37216USA Present address:Trumbull College Yale UniversityNew HavenCT 06511USA

Retinitis pigmentosa is a leading cause of blindness and a progressive retinal disorder,affecting millions of people *** disease is characterized by photoreceptor degeneration,eventually leading to complete *** dominant(adRP)has been associated with mutations in at least four ubiquitously expressed genes encoding pre-mRNA splicing factors—Prp3,Prp8,Prp31 and *** 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 *** investigate the in vivo function of these adRP-associated splicing factor genes,we examined Drosophila in which expression of fly Prp31 homolog was *** 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 *** transgenic DmPrp31RNAi flies provide a useful tool for identifying genetic modifiers or interacting genes for *** of the human Prp31 in these animals leads to a partial rescue of the eye *** results indicate that the Drosophila CG6876 is the fly ortholog of mammalian Prp31 gene.

关键词： Drosophila photoreceptor degeneration retinitis pigmentosa Prp31

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Progranulin promotes neurite outgrowth and neuronal differentiation by regulating GSK-3β

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Protein & Cell 2010年第6期1卷 552-562页

作者： Xue Gao Alvin P.Joselin Lei Wang Amar Kar payal ray Andrew Bateman Alison M.Goate Jane Y.Wu Department of Neurology Lurie Cancer CenterCenter for Genetic Medicine Northwestern University Feinberg School of MedicineChicagoIL 60611USA Division of Experimental Medicine McGill Universityand Endocrine Research LaboratoryRoyal Victoria HospitalMontréalQuébecCanada Department of Psychiatry Washington University School of MedicineSt.LouisMO 63110USA Present address:Cellular and Molecular Medicine University of OttawaOntarioCanada

Progranulin(PGRN)has recently emerged as a key player in a subset of frontotemporal dementias(FTD).Numerous mutations in the progranulin gene have been identified in patients with familial or sporadic frontotemporal lobar degeneration(FTLD).In order to understand the molecular mechanisms by which PGRN deficiency leads to FTLD,we examined activity of PGRN in mouse cortical and hippocampal neurons and in human neuroblastoma SH-SY5Y *** of mouse neurons with PGRN protein resulted in an increase in neurite outgrowth,supporting the role of PGRN as a neurotrophic *** treatment stimulated phosphorylation of glycogen synthase kinase-3 beta(GSK-3β)in cultured *** of PGRN in SH-SY5Y cells impaired retinoic acid induced differentiation and reduced the level of phosphorylated GSK-3β.PGRN knockdown cells were also more sensitized to staurosporineinduced *** results reveal an important role of PGRN in neurite outgrowth and involvement of GSK-3βin mediating PGRN *** of GSK-3βactivation as a downstream event for PGRN signaling provides a mechanistic explanation for PGRN activity in the nervous *** work also suggest that loss of axonal growth stimulation during neural injury repair or deficits in axonal repair may contribute to neuronal damage or axonal loss in FTLD associated with PGRN ***,our study suggests that modulating GSK-3βor similar signaling events may provide therapeutic benefits for FTLD cases associated with PGRN mutations.

关键词： progranulin frontotemporal lobar degeneration glycogen synthase kinase 3 beta(GSK-3β) neurite outgrowth

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Effect of Fe^(3+)Doping Concentration in Cu_(2)ZnSnS_(4) Thin Film:Structural and Optical Analysis

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材料科学与工程（中英文A版） 2022年第3期12卷 97-106页

作者： Shariful Islam payal Majumdar Md.Abul Hossain Farid Ahmed Department of Physics Jahangirnagar UniversitySavarDhaka-1342Bangladesh

CZTS(copper zinc tin sulfide),Cu_(2)ZnSnS_(4),is a potential demanding candidate that is used as absorber layer in solar *** CZTS,Fe^(3+)doped CZTS and CFTS(replacing Zn be Fe^(3+))thin films were deposited on a glass substrate using spin coating *** structural and optical properties of all the thin films were studied to investigate the effect of iron doping in *** XRD(X-ray diffraction)results show that all the thin films grow tetragonal crystal *** crystallite size of the doped CZTS was higher than the pristine CZTS although it was lowest for *** optical band gaps varied from 1.45 eV and 1.83 eV which are suitable for absorbing sunlight.

关键词： CZTS CFTS solar cell absorption coefficient band gap

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