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*** Endocytic Adaptor Protein Numb Functions in Müller Glia to Maintain Retinal Polarity and Photoreceptor Survival through the Polarity Determinant Crumbs

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Annals of Eye Science 2019年第1期 221-221页

作者： Maude Vinette Marie-Claude Bélanger Christine Jolicoeur Mina Gabraie pierre lachapelle Michel Cayouette Cellular Neurobiology Research Unit Institut de recherches cliniques de Montréal(IRCM)MontrealQCCanada Integrated Program in Neuroscience McGill UniversityMontrealQCCanada Division of Experimental Medicine McGill UniversityMontrealQCCanada Department of Ophthalmology Research Institute of the McGill University Health CentreMontrealQCCanada Department of Neurology and Neurosurgery McGill UniversityMontrealQCCanada Department of Medicine Universitéde MontréalMontrealQCCanada

Background:The loss of cell polarity plays a key part in retinal dystrophies such as retinitis pigmentosa(RP)and Leber congenital amaurosis(LCA),resulting in photoreceptor(PR)degeneration and vision *** not knowing the direct genotype-to-phenotype correlation,many disease-causing mutations in the polarity determinant Crumbs(Crb1),have been ***,the loss of Crb1 in mice was shown to cause PR death,due to the loss of adhesions between PR and Müller cells at the apical surface of the ***,although the role of Crb1 in neuron polarity and survival is well established,little is known about how its intracellular trafficking is *** future treatments for retinal degenerative diseases in mind,the goal of this project is to understand the mechanism by which Crb1 is regulated and how it maintains retinal *** work in our laboratory showed that Numb,an endocytic adaptor protein,is an important regulator of protein trafficking in retinal *** therefore hypothesized that Numb might function as regulator of Crb1 in Müller ***:To study Numb function in Müller cells,we generated a conditional knockout(cKO)mouse line to inactivate Numb specifically in Müller cells by crossing a Glast-CreERT2 mouse line with a Numb-floxed *** 30 days,mice were administered tamoxifen to trigger inactivation of Numb and retinas were then collected at time points varying from 2 weeks to 17 months for ***,we studied the retinal morphology and outer limiting membrane integrity by histology and *** electron microscopy(EM),adhesions between Müller glia and photoreceptors were analysed and retinal function was assayed in live mice by electroretinography(ERG).To detect protein expression levels,protein extracts were prepared from cKO and control retinas for *** test for the presence of a biochemical interaction,Hek-293 cells were transfected with Numb and Crb1 vectors,and protein ext

关键词： Degeneration retinal polarity Crumbs-related dystrophies(Crb1-related dystrophies)

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*** in a mouse model for Zellweger spectrum disorder

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Annals of Eye Science 2018年第1期 418-418页

作者： Catherine Argyriou Anna Polosa Bruno Cecyre Erminia Di Pietro Monica Hsieh Jean-Francois Bouchard pierre lachapelle Nancy Braverman Human Genetics McGill UniversityMontrealQCCanada Ophthalomology McGill UniversityMontrealQCCanada Optometry University of MontrealMontrealQCCanada

Background:Zellweger spectrum disorder(ZSD)is an autosomal recessive disease caused by mutations in any one of 13 PEX genes whose protein products are required for peroxisome *** leading to blindness is one of the major untreatable handicaps faced by patients with ZSD but is not well characterized,and the requirement for peroxisomes in retinal health is *** address this and to inform future therapeutic studies,we examined the progression of retinopathy in our murine model for the common PEX1-G843D ***:Retinal electrophysiology(ffERG)and histology were performed in a cohort of Pex1-G844D(equivalent to human G843D)mice from 2 to 32 *** acuity was assessed using *** levels of PEX1-G843D protein,its binding partner Pex6,and its putative ligand Pex5(the peroxisome enzyme receptor)in the retina were determined by *** biochemical metabolites in the whole eye and retina were measured using LC/*** immunohistochemistry was used to visualize various cell ***:Cone ffERG response in the mutants remained residual(5%that of control)regardless of *** rod-mediated responses(50-70%of control)was reached at 4-6 wks,and then progressively decreased with age.B-waves were affected more severely than a-waves,while high frequency ERG components(oscillatory potentials)are better preserved than low frequency components(a-and b-waves).Visual evoked potential was diminished at 32 *** of visual acuity using optokinetics showed low visual reflexes by 11-13 weeks of *** found normal amounts of Pex1-G844D,Pex6,and Pex5 protein in retina,suggesting that the mutated protein is not *** of peroxisome metabolites showed elevated very long chain fatty acids(VLCFA)and decreased plasmalogens in the whole eye,indicative of peroxisome *** the retina,VLCFAs were not elevated,and only C22:6(docosahexaenoic acid)was decreased of the plasmalogens *** were

关键词： Zellweger spectrum disorder(ZSD) peroxisome disorder PEX1

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*** mediated PEX1 gene augmentation improves visual function in a mouse model for Zellweger spectrum disorder

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Annals of Eye Science 2018年第1期 443-443页

作者： Catherine Argyriou Ji Yun Song Ania Polosa Bruno Cecyre Jean-Francois Bouchard pierre lachapelle Jean Bennett Nancy Braverman Human Genetics McGill UniversityMontrealQCCanada Perelman School of Medicine University of PennsylvaniaPhiladelphiaPAUSA Ophthalomology McGill UniversityMontrealQCCanada School of Optometry University of MontrealMontrealQCCanada

Background:Zellweger spectrum disorder(ZSD)is an autosomal recessive disease caused by mutations in any one of 13 PEX genes whose protein products are required for peroxisome *** leading to blindness is one of the major handicaps faced by affected individuals,but treatment for this is supportive *** test whether we could improve visual function in ZSD,we performed a proof-of-concept trial for PEX1 gene augmentation therapy using the Pex1-G844D mouse model,which bears the equivalent to a common human *** model exhibits a gradual decline in scotopic ffERG response,an always residual photopic ffERG response,diminished visual acuity,and cone and bipolar cell ***:We administered subretinal injections of a PEX1-containing viral vector(***)to 2 mouse cohorts of 5 or 9 weeks of age.A GFP-containing vector was used as a control in the contralateral eye of each *** expression of the virus was confirmed by retinal histology/immunohistochemistry,and its ability to recover peroxisome import was confirmed in *** ffERG and optokinetic(OKN)analyses were performed on a subset of animals at 8,16,and 20 weeks after gene *** ffERG and OKN measures were performed when each cohort reached 32 weeks of age(23 or 27 weeks post injection).Results:Preliminary ffERG and OKN analyses at 8 weeks post injection showed mildly better retinal response and visual acuity,respectively,in the PEX1-injected eyes,as did ffERG analysis when each cohort reached 25 weeks of age(16 or 20 weeks after gene delivery).This effect was more pronounced in the cohort treated at 5 weeks of age,when ffERG response is highest in Pex1-G844D *** 32 weeks of age,the ffERG response in the PEX1-injected eyes was double that of GFP-injected eyes,on average,though there was no change in ***,in PEX1-injected eyes the photopic ffERG response improved over time,and the decline in scotopic b-wave amplitude was ameliorat

关键词： Retinal gene therapy peroxisome disorder Zellweger spectrum disorder(ZSD) PEX1

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AB014. What is the role of sildenafil in repairing retinopathy of prematurity?

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Annals of Eye Science 2018年第1期 420-420页

作者： Alexandra Bélanger Shulin Li Annie Poon Suna Jung Palig Balian Zehra Khoja Ania Polosa pierre lachapelle Pia Wintermark Department of Pediatrics Division of Newborn MedicineMontréal Children’s HospitalMcGill UniversityMontréalQCCanada Department of Ophthalmology and Neurology-Neurosurgery Montréal Children’s Hospital-Research InstituteMcGill UniversityMontréalQCCanada

Background:Oxygen therapy provided to support the lungs of premature newborns often leads to damages to the retina called retinopathy of prematurity(ROP)and long-term visual *** treatments for ROP are invasive and aim at preventing further progression of the damages to the retina,but do not repair these *** goal is to investigate the therapeutic effect of sildenafil on retinal structure in a rat model of ***:Sprague-Dawley rats were exposed to hyperoxia(i.e.,80%oxygen)interrupted by three 0.5-hour periods of normoxia(i.e.,21%oxygen)(hyperoxic animals)per day or room air only(i.e.,21%oxygen)(control animals)from post-natal day 4(P4)to 14(P14).Pups were then housed in room ***(50 mg/kg)or vehicle was given per os twice daily after oxygen exposure(from P15 to P21).At P30,retinas were extracted,and *** retinal histology,eyes were stained with toluidine blue to measure the thicknesses of the different retinal *** was also performed to count the number of retinal ganglion cells and bipolar cells in the inner retina,as well as the number of astrocytes and microglia within the different retinal ***:Hyperoxia caused a reduction in thickness of the outer plexiform layer(OPL)and a decrease in the number of bipolar cells in some parts of the retina,compared to control animals(P<0.05);in addition,the number of microglia cells was significantly increased in the rats exposed to hyperoxia,compared to controls(P<0.05).Sildenafil improved OPL thickness in ROP animals,but did not change the number of bipolar *** hyperoxic rats treated with sildenafil,the number of microglia were similar to control *** number of retinal ganglion cells and astrocytes did not differ significantly between the ***:Treatment with sildenafil following oxygen exposure provided some recovery of the structure of the *** beneficial effect may be modulated by a decrease of inflammation within t

关键词： Neonates hyperoxia retinopathy of prematurity(ROP) neuroscience vision health

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AB035. Lactate receptor GPR81 modulates epigenetic modification in the subretina

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Annals of Eye Science 2018年第1期 441-441页

作者： Xiaojuan Yang Raphael Rouget Tang Zhu Christiane Quiniou Shasha Lv Suna Jung Francois Duhamel Houda Tahiri Samy Omri Baraa Noueihed Xin Hou JoséCarlos Rivera Mathieu Nadeau-Vallée pierre lachapelle Sylvain Chemtob Department of Pharmacology McGill UniversityMontréalQCCanada Department of Pharmacology Universitéde MontréalMontréalQCCanada Research Center CHU Sainte-Justine Universitéde MontréalMontréalQCCanada Department of Ophthalmology and Neurology-Neurosurgery Montréal Children’s Hospital-Research InstituteMcGill UniversityMontréalQCCanada Research Center Hôpital Maisonneuve-Rosemont Universitéde MontréalMontréalQCCanada Department of Ophthalmology Universitéde MontréalMontréalQCCanada Department of Pediatrics Universitéde MontréalMontréalQCCanada

Background:Retinal pigment epithelium(RPE)is vital for the homeostasis of the subretina including photoreceptors and ***,our previous results suggested that the recently discovered lactate receptor GPR81 is abundantly expressed in *** date,only one previous study has shown that activating GPR81 could enhance DNA repair by activating ***,we investigated whether GPR81 exhibits epigenetic modification in the subretina by using GPR81−/−***:GPR81−/−mice and wide type littermates were generated on a background of C57BL/6J *** thicknesses of their choroid were evaluated by ***,Q-PCR,western blot and choroid sprout assay were *** vitro,primary retinal pigment epithelium(pRPE)cells were isolated from mice,and cultured for ***:The thickness of choroid was reduced in GPR81−/−mice compared to GPR81+/+mice,suggesting that GPR81 is important for the integrity of *** the choroid sprout assay,lactate treated RPE/choroid complex showed a significant increase in angiogenesis compared to controls while lactate treated KO RPE/choroid complex showed no difference compared to their *** Q-PCR,most of the genes screened elevated their expression in GPR81−/−mice compared to WT mice,suggesting epigenetic modification may exist,which were confirmed by histone acetylation and HDACs activity ***:Taking together,the lactate receptor GPR81 in RPE is very important for maintaining homeostasis of the *** novel discovery sheds new light on the relationship between metabolism and epigenetic modification.

关键词： Subretina GPR81 epigenetic modification

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Diesel engine exhaust exposures in two underground mines

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International Journal of Mining Science and Technology 2017年第4期27卷 641-645页

作者： Debia Maximilien Couture Caroline Njanga pierre-Eric Neesham-Grenon Eve lachapelle Guillaume Coulombe Hugo Hallé Stéphane Aubin Simon Department of Environmental and Occupational Health School of Public Health Universite de Montreal 2375 chemin de la Cote Ste-Catherine Montreal H3T IA8 Canada Department of Mechanical Engineering F.cole de Technologie Superieure 1100 rue Notre-Dame Ouest Montreal H3C 1K3 Canada Health and Safety Agnico Eagie Mines 145 King Street East Suite 400 Toronto MSC 2Y7 Canada Health and Safety Westwood Mine (IAMGOLD) Chemin Arthur Doyon PreissacJOY 2EO Canada Institut de recherche Robert-Sauve en sante et en securite du travail (IRSST) 505 Boul. de Maisonneuve Ouest Montreal H3A 3C2 Canada

Exposure to diesel engine exhaust(DE) is a major concern in underground mines. It has been linked to cardiopulmonary diseases and is classified as a human carcinogen. The goal of this study is to assess DE exposures in workers at two underground gold mines, to compare exposure levels within and between the mines, and to compare different methods of measuring DE exposures, namely respirable combustible dust(RCD), elemental carbon(EC) and total carbon(TC). Ambient and personal breathing zone(PBZ) measurements were taken. Side-by-side monitoring of RCD and of the respirable fraction of EC and TC(EC_Rand TC_R) was carried out in the workers' breathing zone during full-shift *** ambient measurements, in addition to EC_R, TC_Rand RCD, a submicron aerosol fraction(less than 1 mm) of EC and TC was also sampled(EC_1and TC_1). Average ambient results of 240 mg/m^3 in RCD, 150 mg/m^3 in EC_Rand 210 mg/m^3 in TC_Rare obtained. Average PBZ results of 190 mg/m^3 in RCD,84 mg/m^3 in EC3Rand 150 mg/min TC_Rare obtained. Very good correlation is found between EC_Rand EC_1 with a Pearson correlation coefficient of 0.99(p < 0.01) calculated between the two logtransformed concentrations. No differences are reported between EC_Rand EC_1, nor between TC_Rand TC_1, since ratios are equal to 1.04, close to 1, in both cases. Highest exposures are reported for loadhaul-dump(LHD) and jumbo drill operators and conventional miners. Significant exposure differences are reported between mines for truck and LHD operators(p < 0.01). The average TC_R/EC_Rratio is 1.6 for PBZ results, and 1.3 for ambient results. The variability observed in the TC_R/EC_Rratio shows that interferences from non-diesel related organic carbon can skew the interpretation of results when relying only on TC data.

关键词： Diesel exposure Underground mine Respirable combustible dust Elemental carbon Total carbon Diesel particulate matter Similar exposure groups

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