RESUMEN
Rod photoreceptors contribute to vision over an â¼ 6-log-unit range of light intensities. The wide dynamic range of rod vision is thought to depend upon light intensity-dependent switching between two parallel pathways linking rods to ganglion cells: a rod â rod bipolar (RB) cell pathway that operates at dim backgrounds and a rod â cone â cone bipolar cell pathway that operates at brighter backgrounds. We evaluated this conventional model of rod vision by recording rod-mediated light responses from ganglion and AII amacrine cells and by recording RB-mediated synaptic currents from AII amacrine cells in mouse retina. Contrary to the conventional model, we found that the RB pathway functioned at backgrounds sufficient to activate the rod â cone pathway. As background light intensity increased, the RB's role changed from encoding the absorption of single photons to encoding contrast modulations around mean luminance. This transition is explained by the intrinsic dynamics of transmission from RB synapses.
Asunto(s)
Adaptación Ocular/fisiología , Células Bipolares de la Retina/fisiología , Células Fotorreceptoras Retinianas Bastones/fisiología , Sinapsis/fisiología , 2-Amino-5-fosfonovalerato/farmacología , Adaptación Ocular/efectos de los fármacos , Animales , Biofisica , Simulación por Computador , Antagonistas de Aminoácidos Excitadores/farmacología , Glucosamina 6-Fosfato N-Acetiltransferasa/deficiencia , Glucosamina 6-Fosfato N-Acetiltransferasa/genética , Proteínas Fluorescentes Verdes/genética , Proteínas Fluorescentes Verdes/metabolismo , Técnicas In Vitro , Luz , Ratones , Ratones Endogámicos C57BL , Ratones Noqueados , Ratones Transgénicos , Modelos Neurológicos , Técnicas de Placa-Clamp , Quinoxalinas/farmacología , Células Fotorreceptoras Retinianas Bastones/efectos de los fármacos , Sinapsis/efectos de los fármacos , Vías Visuales/fisiologíaRESUMEN
Ether-phospholipids represent an important group of phospholipids characterized by an alkyl or an alkenyl bond at the sn-1 position of the glycerol backbone. Plasmalogens are the most abundant form of alkenyl-glycerophospholipids, and their synthesis requires functional peroxisomes. Defects in the biosynthesis of plasmalogens are the biochemical hallmark of the human peroxisomal disorder Rhizomelic Chondrodysplasia Punctata (RCDP), which is characterized by defects in eye, bone and nervous tissue. The generation and characterization of mouse models with defects in plasmalogen levels have significantly advanced our understanding of the role and importance of plasmalogens as well as pathogenetic mechanisms underlying RCDP. A review of the current mouse models and the description of the combined knowledge gathered from the histopathological and biochemical studies is presented and discussed. Further characterization of the role and functions of plasmalogens will contribute to the elucidation of disease pathogenesis in peroxisomal and non-peroxisomal disorders. This article is part of a Special Issue entitled: Metabolic Functions and Biogenesis of Peroxisomes in Health and Disease.