Search

Your search keyword '"Sahly, Iman"' showing total 26 results
Start Over Author "Sahly, Iman"
26 results on '"Sahly, Iman"'

10. Cone degeneration is triggered by the absence of USH1 proteins but prevented by antioxidant treatments

Author

Trouillet, Alix, Dubus, Elisabeth, Degardin, Julie, Estivalet, Amrit, Ivkovic, Ivana, Godefroy, David, García-Ayuso, Diego, Simonutti, Manuel, Sahly, Iman, Sahel, José-Alain, El-Amraoui, Aziz, Petit, Christine, Picaud, Serge, Institut de la Vision, Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Sorbonne Université-Centre National de la Recherche Scientifique ( CNRS ), Universidad de Murcia, Génétique et Physiologie de l'Audition, Collège de France ( CdF ) -Institut Pasteur [Paris]-Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Sorbonne Université, CIC Quinze-Vingts, Assistance publique - Hôpitaux de Paris (AP-HP)-Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Centre Hospitalier National d'Ophtalmologie des Quinze-Vingts, Fondation Ophtalmologique Adolphe de Rothschild [Paris], Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS), Institut Pasteur [Paris] (IP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU), Académie des Sciences [Paris], Institut de France, Centre d'investigation clinique Quinze-Vingts [CHNO] (CIC1423 - CIC QUINZE-VINGTS), Institut Hospitalo-Universitaire FOReSIGHT, Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre Hospitalier National d'Ophtalmologie des Quinze-Vingts (CHNO)-Sorbonne Université (SU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre Hospitalier National d'Ophtalmologie des Quinze-Vingts (CHNO)-Sorbonne Université (SU), Collège de France (CdF (institution)), This work was supported by EC-FP7 TREATRUSH (HEALTH-F2-2010-242013), by the LabEx LIFESENSES (ANR-10-LABX-65), managed by the French Agence National pour la Recherche (ANR) as part of the first Investissements d’Avenir program (ANR-11-IDEX-0004-02), and within the second Investissements d’Avenir program (ANR-15-RHUS-0001), and by the Faun stiftung and RHU Light4Deaf., ANR-11-IDEX-0004,SUPER,Sorbonne Universités à Paris pour l'Enseignement et la Recherche(2011), ANR-15-RHUS-0001,LIGHT4DEAF,ECLAIRER LA SURDITÉ : UNE APPROCHE HOLISTIQUE DU SYNDROME D'USHER(2015), European Project: 242013,EC:FP7:HEALTH,FP7-HEALTH-2009-single-stage,TREATRUSH(2010), HAL UPMC, Gestionnaire, Sorbonne Universités à Paris pour l'Enseignement et la Recherche - - SUPER2011 - ANR-11-IDEX-0004 - IDEX - VALID, ECLAIRER LA SURDITÉ : UNE APPROCHE HOLISTIQUE DU SYNDROME D'USHER - - LIGHT4DEAF2015 - ANR-15-RHUS-0001 - RHUS - VALID, Fighting blindness of Usher syndrome: diagnosis, pathogenesis and retinal treatment (TreatRetUsher) - TREATRUSH - - EC:FP7:HEALTH2010-02-01 - 2014-01-31 - 242013 - VALID, Institut Hospitalo-Universitaire FOReSIGHT (IHU FOReSIGHT), Centre National de la Recherche Scientifique (CNRS)-Sorbonne Université (SU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Chaire Génétique et physiologie cellulaire, Centre Hospitalier National d'Ophtalmologie des Quinze-Vingts (CHNO), Académie des Sciences, and UMRS 1120, Institut National de la Santé et de la Recherche Médicale de Paris

Subjects
genetic structures, Taurine, lcsh:Medicine, Apoptosis, Cell Cycle Proteins, Nerve Tissue Proteins, Article, Antioxidants, Glial Fibrillary Acidic Protein, otorhinolaryngologic diseases, Animals, Gliosis, [SDV.MHEP.OS]Life Sciences [q-bio]/Human health and pathology/Sensory Organs, lcsh:Science, Mice, Inbred BALB C, Opsins, [ SDV ] Life Sciences [q-bio], Retinal Degeneration, lcsh:R, Darkness, Housing, Animal, eye diseases, Diet, Mice, Inbred C57BL, Cytoskeletal Proteins, Phenotype, [SDV.MHEP.OS] Life Sciences [q-bio]/Human health and pathology/Sensory Organs, Retinal Cone Photoreceptor Cells, lcsh:Q, sense organs, Carrier Proteins
Abstract

International audience; Usher syndrome type 1 (USH1) is a major cause of inherited deafness and blindness in humans. The eye disorder is often referred to as retinitis pigmentosa, which is characterized by a secondary cone degeneration following the rod loss. The development of treatments to prevent retinal degeneration has been hampered by the lack of clear evidence for retinal degeneration in mutant mice deficient for the Ush1 genes, which instead faithfully mimic the hearing deficit. We show that, under normal housing conditions, Ush1g−/− and Ush1c−/− albino mice have dysfunctional cone photoreceptors whereas pigmented knockout animals have normal photoreceptors. The key involvement of oxidative stress in photoreceptor apoptosis and the ensued retinal gliosis were further confirmed by their prevention when the mutant mice are reared under darkness and/or supplemented with antioxidants. The primary degeneration of cone photoreceptors contrasts with the typical forms of retinitis pigmentosa. Altogether, we propose that oxidative stress probably accounts for the high clinical heterogeneity among USH1 siblings, which also unveils potential targets for blindness prevention.

Published
2018
Full Text
View/download PDF

11. Localization of Usher 1 proteins to the photoreceptor calyceal processes, which are absent from mice.: Usher 1 retinal pathogenesis

Author

Sahly, Iman, Dufour, Eric, Schietroma, Cataldo, Michel, Vincent, Bahloul, Amel, Perfettini, Isabelle, Pepermans, Elise, Estivalet, Amrit, Carette, Diane, Aghaie, Asadollah, Ebermann, Inga, Lelli, Andrea, Iribarne, Maria, Hardelin, Jean-Pierre, Weil, Dominique, Sahel, José-Alain, El-Amraoui, Aziz, Petit, Christine, Institut de la Vision, Centre National de la Recherche Scientifique (CNRS)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Pierre et Marie Curie - Paris 6 (UPMC), Chaire Génétique et physiologie cellulaire, Collège de France (CdF (institution)), European Union Seventh Framework Program, under grant agreement HEALTH-F2-2010-242013 (TREATRUSH), LHW-Stiftung, Fondation Raymonde & Guy Strittmatter, Fighting Blindness, FAUN Stiftung (Suchert Foundation), Conny Maeva Charitable Foundation, Fondation Orange, ERC grant 294570-hairbundle, and Louis-Jeantet Foundation (C. Petit), French State program 'Investissements d'Avenir' managed by the Agence Nationale de la Recherche (grant reference: ANR-10-LBX-65), Foundation Fighting Blindness Paris Center Grant and Fondation Voir et Entendre (C. Petit and J.-A. Sahel), and ANR-07-MRARE-009-01 grant to A. El-Amraoui., Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), and Collège de France - Chaire Génétique et physiologie cellulaire

Subjects
adhesion, otorhinolaryngologic diseases, photoreceptor cells, outer segment, sense organs, [SDV.MHEP.OS]Life Sciences [q-bio]/Human health and pathology/Sensory Organs, Usher syndrome, calyceal processes
Abstract

International audience; The mechanisms underlying retinal dystrophy in Usher syndrome type I (USH1) remain unknown because mutant mice lacking any of the USH1 proteins-myosin VIIa, harmonin, cadherin-23, protocadherin-15, sans-do not display retinal degeneration. We found here that, in macaque photoreceptor cells, all USH1 proteins colocalized at membrane interfaces (i) between the inner and outer segments in rods and (ii) between the microvillus-like calyceal processes and the outer segment basolateral region in rods and cones. This pattern, conserved in humans and frogs, was mediated by the formation of an USH1 protein network, which was associated with the calyceal processes from the early embryonic stages of outer segment growth onwards. By contrast, mouse photoreceptors lacked calyceal processes and had no USH1 proteins at the inner-outer segment interface. We suggest that USH1 proteins form an adhesion belt around the basolateral region of the photoreceptor outer segment in humans, and that defects in this structure cause the retinal degeneration in USH1 patients.

Published
2012
Full Text
View/download PDF

12. A subpopulation of serotonergic neurons that do not express the 5-HT1A autoreceptor.

Author

Kiyasova, Vera, Bonnavion, Patricia, Scotto-Lomassese, Sophie, Fabre, Véronique, Sahly, Iman, Tronche, François, Deneris, Evan, Gaspar, Patricia, Fernandez, Sebastian P, Kiyasova, Vera, Bonnavion, Patricia, Scotto-Lomassese, Sophie, Fabre, Véronique, Sahly, Iman, Tronche, François, Deneris, Evan, Gaspar, Patricia, and Fernandez, Sebastian P

Abstract

5-HT neurons are topographically organized in the hindbrain, and have been implicated in the etiology and treatment of psychiatric diseases such as depression and anxiety. Early studies suggested that the raphe 5-HT neurons were a homogeneous population showing similar electrical properties, and feedback inhibition mediated by 5-HT1A autoreceptors. We utilized histochemistry techniques in ePet1-eGFP and 5-HT1A-iCre/R26R mice to show that a subpopulation of 5-HT neurons do not express the somatodendritic 5-HT1A autoreceptor mRNA. In addition, we performed patch-clamp recordings followed by single-cell PCR in ePet1-eGFP mice. From 134 recorded 5-HT neurons located in the dorsal, lateral, and median raphe, we found lack of 5-HT1A mRNA expression in 22 cells, evenly distributed across raphe subfields. We compared the cellular characteristics of these neuronal types and found no difference in passive membrane properties and general excitability. However, when injected with large depolarizing current, 5-HT1A-negative neurons fired more action potentials, suggesting a lack of autoinhibitory action of local 5-HT release. Our results support the hypothesis that the 5-HT system is composed of subpopulations of serotonergic neurons with different capacity for adaptation., info:eu-repo/semantics/published

Published
2013

18. A human homologue of the Drosophila eyes absent gene underlies Branchio-Oto-Renal (BOR) syndrome and identifies a novel gene family

Author

Abdelhak, Sonia, primary, Kalatzis, Vasiliki, additional, Heilig, Roland, additional, Compain, Sylvie, additional, Samson, Delphine, additional, Vincent, Christophe, additional, Weil, Dominique, additional, Cruaud, Corinne, additional, Sahly, Iman, additional, Leibovici, Michel, additional, Bitner-Glindzicz, Maria, additional, Francis, Mary, additional, Lacombe, Didier, additional, Vigneron, Jacqueline, additional, Charachon, Robert, additional, Boven, Katia, additional, Bedbeder, Philippe, additional, Regemorter, Nicole Van, additional, Weissenbach, Jean, additional, and Petit, Christine, additional

Published
1997
Full Text
View/download PDF

20. Syndrome de Usher de type IB : Anomalie d'une myosine non conventionnelle

Author

Weil, Dominique, primary, Levy, Gallia, additional, Sahly, Iman, additional, Lévi-Acobas, Fabienne, additional, Blanchard, Stéphane, additional, Crozet, Fabien, additional, Kaplan, Josseline, additional, Munnich, Arnold, additional, Abitbol, Marc, additional, Steel, Karen, additional, Brown, Steve, additional, and Petit, Christine, additional

Published
1995
Full Text
View/download PDF

24. <TOGGLE>Eya1</TOGGLE> expression in the developing ear and kidney: Towards the understanding of the pathogenesis of branchio-oto-renal (BOR) syndrome

Author

Kalatzis, Vasiliki, Sahly, Iman, El-Amraoui, Aziz, and Petit, Christine

Abstract

Branchio-Oto-Renal (BOR) syndrome is an autosomal dominant, early developmental defect characterised by varying combinations of branchial (fistulas, sinuses, and cysts), outer, middle and inner ear, and renal anomalies. The gene underlying this syndrome, EYA1, is homologous to the Drosophila developmental gene eyes absent which encodes a transcriptional co-activator required for eye specification. We report here the temporal and spatial pattern of expression of the murine homologue, Eya1, throughout ear and kidney development in relation to the anomalies of BOR syndrome. The expression of Eya1 in the branchial arch apparatus (namely in the 2nd, 3rd, and 4th branchial clefts and pharyngeal pouches) at embryonic day (E)10.5, can be correlated with the branchial fistulas, sinuses, and cysts but not with the outer and middle ear anomalies. In contrast, Eya1 is expressed during the slightly more advanced stage of outer and middle ear morphogenesis at E13.5, in the mesenchyme adjacent to the first branchial cleft (the cleft will give rise to the external auditory canal and the surrounding mesenchyme to the auricular hillocks) and surrounding the primordia of the middle ear ossicles, and in the epithelium of the tubotympanic recess (the future tympanic cavity). During early inner ear development, Eya1 is expressed in the ventromedial wall of the otic vesicle (the site of the future sensory epithelia), in the statoacoustic ganglion, and in the periotic mesenchyme, consistent with the cochlear anomalies and sensorineural hearing loss of BOR syndrome. Subsequently, Eya1 expression is observed in the differentiating hair and supporting cells of the sensory epithelia, as well as in the associated ganglia, and persists after differentiation has taken place. This suggests that, in addition to a role in the morphogenetic process, Eya1 could also be implicated in the differentiation and/or survival of these inner ear cell populations. Finally, Eya1 expression in the condensing mesenchymal cells of the kidney is consistent with the excretory and collecting system anomalies of BOR syndrome. From the comparison of the Eya1 and Pax2 expression patterns during ear and kidney development, a contribution of these two genes to the same regulatory pathway can only be suggested in the mesenchymal-epithelial transition directing renal tubule formation. Dev. Dyn. 1998;213:486–499. © 1998 Wiley-Liss, Inc.

Published
1998
Full Text
View/download PDF

25. Cone degeneration is triggered by the absence of USH1 proteins but prevented by antioxidant treatments.

Author

Trouillet A, Dubus E, Dégardin J, Estivalet A, Ivkovic I, Godefroy D, García-Ayuso D, Simonutti M, Sahly I, Sahel JA, El-Amraoui A, Petit C, and Picaud S

Subjects
Animals, Antioxidants pharmacology, Apoptosis, Cell Cycle Proteins, Cytoskeletal Proteins, Darkness, Diet, Glial Fibrillary Acidic Protein metabolism, Gliosis pathology, Housing, Animal, Mice, Inbred BALB C, Mice, Inbred C57BL, Opsins metabolism, Phenotype, Retinal Cone Photoreceptor Cells drug effects, Retinal Degeneration pathology, Taurine administration & dosage, Antioxidants therapeutic use, Carrier Proteins metabolism, Nerve Tissue Proteins metabolism, Retinal Cone Photoreceptor Cells pathology, Retinal Degeneration drug therapy, Retinal Degeneration prevention & control
Abstract

Usher syndrome type 1 (USH1) is a major cause of inherited deafness and blindness in humans. The eye disorder is often referred to as retinitis pigmentosa, which is characterized by a secondary cone degeneration following the rod loss. The development of treatments to prevent retinal degeneration has been hampered by the lack of clear evidence for retinal degeneration in mutant mice deficient for the Ush1 genes, which instead faithfully mimic the hearing deficit. We show that, under normal housing conditions, Ush1g -/- and Ush1c -/- albino mice have dysfunctional cone photoreceptors whereas pigmented knockout animals have normal photoreceptors. The key involvement of oxidative stress in photoreceptor apoptosis and the ensued retinal gliosis were further confirmed by their prevention when the mutant mice are reared under darkness and/or supplemented with antioxidants. The primary degeneration of cone photoreceptors contrasts with the typical forms of retinitis pigmentosa. Altogether, we propose that oxidative stress probably accounts for the high clinical heterogeneity among USH1 siblings, which also unveils potential targets for blindness prevention.

Published
2018
Full Text
View/download PDF

26. A subpopulation of serotonergic neurons that do not express the 5-HT1A autoreceptor.

Author

Kiyasova V, Bonnavion P, Scotto-Lomassese S, Fabre V, Sahly I, Tronche F, Deneris E, Gaspar P, and Fernandez SP

Subjects
Animals, Electrophysiological Phenomena physiology, Immunohistochemistry, Mice, Mice, Inbred Strains, Raphe Nuclei metabolism, Autoreceptors metabolism, Receptors, Serotonin, 5-HT1 metabolism, Serotonergic Neurons metabolism
Abstract

5-HT neurons are topographically organized in the hindbrain, and have been implicated in the etiology and treatment of psychiatric diseases such as depression and anxiety. Early studies suggested that the raphe 5-HT neurons were a homogeneous population showing similar electrical properties, and feedback inhibition mediated by 5-HT1A autoreceptors. We utilized histochemistry techniques in ePet1-eGFP and 5-HT1A-iCre/R26R mice to show that a subpopulation of 5-HT neurons do not express the somatodendritic 5-HT1A autoreceptor mRNA. In addition, we performed patch-clamp recordings followed by single-cell PCR in ePet1-eGFP mice. From 134 recorded 5-HT neurons located in the dorsal, lateral, and median raphe, we found lack of 5-HT1A mRNA expression in 22 cells, evenly distributed across raphe subfields. We compared the cellular characteristics of these neuronal types and found no difference in passive membrane properties and general excitability. However, when injected with large depolarizing current, 5-HT1A-negative neurons fired more action potentials, suggesting a lack of autoinhibitory action of local 5-HT release. Our results support the hypothesis that the 5-HT system is composed of subpopulations of serotonergic neurons with different capacity for adaptation.

Published
2013
Full Text
View/download PDF

Catalog

Books, media, physical & digital resources
See catalog results