Dystrophin Is Required for Proper Functioning of Luminance and Red-Green Cone Opponent Mechanisms in the Human Retina

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Salgueiro Barboni, Mirella Telles ; Gomes Martins, Cristiane Maria ; Nagy, Balazs Vince ; Tsai, Tina ; Damico, Francisco Max ; da Costa, Marcelo Fernandes ; Pavanello, Rita de Cassia M. ; Vilaca Lourenco, Naila Cristina ; Pereirade Cerqueira, Antonia Maria ; Zatz, Mayana ; Kremers, Jan ; Ventura, Dora Fix

• Document type: Journal article
• Source: INVESTIGATIVE OPHTHALMOLOGY & VISUAL SCIENCE; v. 57, n. 8, p. 3581-3587, JUL 2016.
• Web of Science Citations: 1

Abstract

PURPOSE. Visual information is processed in parallel pathways in the visual system. Parallel processing begins at the synapse between the photoreceptors and their postreceptoral neurons in the human retina. The integrity of this first neural connection is vital for normal visual processing downstream. Of the numerous elements necessary for proper functioning of this synaptic contact, dystrophin proteins in the eye play an important role. Deficiency of muscle dystrophin causes Duchenne muscular dystrophy (DMD), an X-linked disease that affects muscle function and leads to decreased life expectancy. In DMD patients, postreceptoral retinal mechanisms underlying scotopic and photopic vision and ON-and OFF-pathway responses are also altered. METHODS. In this study, we recorded the electroretinogram (ERG) while preferentially activating the (red-green) opponent or the luminance pathway, and compared data from healthy participants (n = 16) with those of DMD patients (n = 10). The stimuli were heterochromatic sinusoidal modulations at a mean luminance of 200 cd/m(2). The recordings allowed us also to analyze ON and OFF cone-driven retinal responses. RESULTS. We found significant differences in 12-Hz response amplitudes and phases between controls and DMD patients, with conditions with large luminance content resulting in larger response amplitudes in DMD patients compared to controls, whereas responses of DMD patients were smaller when pure chromatic modulation was given. CONCLUSIONS. The results suggest that dystrophin is required for the proper function of luminance and red-green cone opponent mechanisms in the human retina. (AU)

• FAPESP's process: 14/06457-5 - Scientific instrumentation in vision research studies at the Vision Laboratory of IPUSP
• Grantee: Dora Selma Fix Ventura
• Support type: Research Grants - Visiting Researcher Grant - International
• FAPESP's process: 12/01115-3 - Development and application of electroretinographic and psychophysical methods for the assessment of on and off mechanisms in both chromatic and luminance visual pathways in Diabetes Mellitus and Duchenne muscular dystrophy
• Grantee: Dora Selma Fix Ventura
• Support type: Regular Research Grants
• FAPESP's process: 13/08028-1 - CEGH-CEL - Human Genome and Stem Cell Research Center
• Grantee: Mayana Zatz
• Support type: Research Grants - Research, Innovation and Dissemination Centers - RIDC
• FAPESP's process: 12/51299-3 - International joint research on the development and application of electroretinographic and psychophysical methods for the assessment of postreceptoral on-and off-mechanisms in both chromatic and luminance retinal visual pathways in Duchenne muscular dystrophy and in Diabetes mellitus
• Grantee: Dora Selma Fix Ventura
• Support type: Regular Research Grants
• FAPESP's process: 14/26818-2 - Development and implementation of visual evaluation methods: clinical applications and animal models
• Grantee: Dora Selma Fix Ventura
• Support type: Research Projects - Thematic Grants