title: The retina of the PCD/PCD mouse as a model of photoreceptor degeneration. A structural and functional study.
abstract: In this work, we used the pcd (Purkinje cell degeneration) mutant mouse with a slow temporal progression of photoreceptor degeneration in order to analyze the structural and functional modifications in the neuronal populations of the outer and inner retina. Retinal immunocytochemistry and functional electroretinography were performed on the pcd/pcd mutant mice and control wild type animals of the C57/DBA strain at 45, 90, 180 and 270 post-natal days. Immunohistochemical studies were performed for a series of protein markers: calbindin, calretinin, PKCα, bassoon, synapsin, syntaxin and islet1. Full field electroretinography recordings were performed on control and dystrophic mice. Rod and mixed responses, and oscillatory potentials, were recorded in dark adapted conditions; cone and flicker responses were recorded under light adaptation. Our results show significant structural modifications in the photoreceptor populations and neurons of the inner retina. Changes in cell morphology affect mainly to the bipolar cells, which gradually lose their dendritic tufts. The electroretinography records reveal that in the pcd retinas the rod and cone systems show a reduction in the amplitude of the electrical signals. This decrease progresses slowly with the passage of time, although for the most advanced stage of photoreceptor degeneration considered, 270 post-natal days, it is still possible to record light induced responses. We conclude that pcd mice experience a loss of retinal function in correlation with the loss of photoreceptors with age, and significant changes in retinal synaptic processes.
authors.affiliation: Department of Physiology, School of Medicine, University of Alcalá, Alcalá de Henares, Madrid, Spain. mmarchena@usal.es
authors.forename: Miguel
authors.initials: M
authors.lastname: Marchena
authors.forename: Juan
authors.initials: J
authors.lastname: Lara
authors.forename: José
authors.initials: J
authors.lastname: Aijón
authors.forename: Francisco
authors.initials: F
authors.lastname: Germain
authors.forename: Pedro
authors.initials: P
authors.lastname: de la Villa
authors.forename: Almudena
authors.initials: A
authors.lastname: Velasco
mesh_terms: D000818:Animals; D015415:Biomarkers / Q000378:metabolism; D003623:Dark Adaptation; D004195:Disease Models, Animal; D004596:Electroretinography; D005260:Female; D007150:Immunohistochemistry; D008297:Male; D051379:Mice; D008810:Mice, Inbred C57BL; D008811:Mice, Inbred DBA; D008817:Mice, Mutant Strains; D010775:Photic Stimulation; D020419:Photoreceptor Cells, Vertebrate / Q000473:pathology*; D016133:Polymerase Chain Reaction; D011689:Purkinje Cells / Q000473:pathology*; D012160:Retina / Q000503:physiopathology*; D058499:Retinal Dystrophies / Q000473:pathology*; D012165:Retinal Ganglion Cells / Q000473:pathology*
chemical_list: D015415:Biomarkers