title: Dystrophin Is Required for Proper Functioning of Luminance and Red-Green Cone Opponent Mechanisms in the Human Retina.
abstract: 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.
  
  
  
  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/m2. The recordings allowed us also to analyze ON and OFF cone-driven retinal responses.
  
  
  
  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.
  
  
  
  The results suggest that dystrophin is required for the proper function of luminance and red-green cone opponent mechanisms in the human retina.
authors.affiliation: Núcleo de Neurociências e Comportamento, Universidade de São Paulo, São Paulo, Brasil 2Departamento de Psicologia Experimental, Instituto de Psicologia, Universidade de São Paulo, São Paulo, Brasil.
authors.forename: Mirella Telles Salgueiro
authors.initials: MT
authors.lastname: Barboni
authors.affiliation: Núcleo de Neurociências e Comportamento, Universidade de São Paulo, São Paulo, Brasil 2Departamento de Psicologia Experimental, Instituto de Psicologia, Universidade de São Paulo, São Paulo, Brasil.
authors.forename: Cristiane Maria Gomes
authors.initials: CM
authors.lastname: Martins
authors.affiliation: Núcleo de Neurociências e Comportamento, Universidade de São Paulo, São Paulo, Brasil 2Departamento de Psicologia Experimental, Instituto de Psicologia, Universidade de São Paulo, São Paulo, Brasil.
authors.forename: Balázs Vince
authors.initials: BV
authors.lastname: Nagy
authors.affiliation: Department of Ophthalmology, University Hospital Erlangen, Erlangen, Germany.
authors.forename: Tina
authors.initials: T
authors.lastname: Tsai
authors.affiliation: Núcleo de Neurociências e Comportamento, Universidade de São Paulo, São Paulo, Brasil 2Departamento de Psicologia Experimental, Instituto de Psicologia, Universidade de São Paulo, São Paulo, Brasil 4Departamento de Oftalmologia, Faculdade de Medicina, Uni.
authors.forename: Francisco Max
authors.initials: FM
authors.lastname: Damico
authors.affiliation: Núcleo de Neurociências e Comportamento, Universidade de São Paulo, São Paulo, Brasil 2Departamento de Psicologia Experimental, Instituto de Psicologia, Universidade de São Paulo, São Paulo, Brasil.
authors.forename: Marcelo Fernandes
authors.initials: MF
authors.lastname: da Costa
authors.forename: Rita
authors.initials: R
authors.lastname: de Cassia
authors.affiliation: Centro de Pesquisas do Genoma Humano e Células-Tronco Universidade de São Paulo, São Paulo, Brasil.
authors.forename: M
authors.initials: M
authors.lastname: Pavanello
authors.affiliation: Centro de Pesquisas do Genoma Humano e Células-Tronco Universidade de São Paulo, São Paulo, Brasil.
authors.forename: Naila Cristina Vilaça
authors.initials: NC
authors.lastname: Lourenço
authors.affiliation: Centro de Pesquisas do Genoma Humano e Células-Tronco Universidade de São Paulo, São Paulo, Brasil.
authors.forename: Antonia Maria Pereira
authors.initials: AM
authors.lastname: de Cerqueira
authors.affiliation: Centro de Pesquisas do Genoma Humano e Células-Tronco Universidade de São Paulo, São Paulo, Brasil.
authors.forename: Mayana
authors.initials: M
authors.lastname: Zatz
authors.affiliation: Department of Ophthalmology, University Hospital Erlangen, Erlangen, Germany.
authors.forename: Jan
authors.initials: J
authors.lastname: Kremers
authors.affiliation: Núcleo de Neurociências e Comportamento, Universidade de São Paulo, São Paulo, Brasil 2Departamento de Psicologia Experimental, Instituto de Psicologia, Universidade de São Paulo, São Paulo, Brasil.
authors.forename: Dora Fix
authors.initials: DF
authors.lastname: Ventura
mesh_terms: D000293:Adolescent; D000328:Adult; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D003118:Color Perception / Q000235:genetics / Q000502:physiology*; D016189:Dystrophin / Q000172:deficiency / Q000235:genetics / Q000502:physiology*; D004596:Electroretinography; D005260:Female; D006801:Humans; D008297:Male; D020388:Muscular Dystrophy, Duchenne / Q000235:genetics / Q000503:physiopathology*; D012160:Retina / Q000502:physiology*; D017949:Retinal Cone Photoreceptor Cells / Q000502:physiology; D055815:Young Adult
chemical_list: D016189:Dystrophin