Papers by Vittorio Porciatti
Investigative Ophthalmology & Visual Science
Electroretinogram (ERG) responses to alternating gratings have been recorded in patients with tem... more Electroretinogram (ERG) responses to alternating gratings have been recorded in patients with temporary occlusion of the retinal artery, retrobulbar optic neuritis, and other ganglion cell diseases. The ERG responses to alternating gratings were absent in first case and dramatically depressed in the others, whereas the ERG responses to light flashes or to homogeneous flickering light were normal. These findings corroborate recent evidence from studies on the cat, showing that the ERG in response to alternating gratings is correlated with ganglion cell activity.
Electroencephalography and Clinical Neurophysiology, 1997
Electroencephalography and Clinical Neurophysiology, 1996
The Changing Visual System, 1991

Investigative ophthalmology & visual science, 2014
To characterize age-related changes of retinal ganglion cell (RGC) function, IOP, and anatomical ... more To characterize age-related changes of retinal ganglion cell (RGC) function, IOP, and anatomical markers of axon/glia integrity in a transgenic mouse expressing Tyr437His mutant of human myocilin protein. Retinal ganglion cell electrical responsiveness was tested with pattern electroretinogram (PERG) in 11 transgenic mice expressing mutated myocilin at different ages over 18 months under ketamine/xylazine anesthesia. Twelve age-matched C57BL/6J mice also were tested as controls. Intraocular pressure was measured with a Tonolab tonometer. Immunohistochemistry for GFAP and neurofilament was performed on dissected optic nerve heads. In transgenic mice expressing mutated myocilin, the PERG amplitude progressively decreased with increasing age by approximately 50%, whereas the PERG peak latency increased by approximately 40 ms (ANOVA, P < 0.05). In contrast, PERGs of young and old control mice had similar amplitudes and peak latencies. In transgenic mice, GFAP staining was more intens...

Molecular vision, 2010
To characterize differences in retinal ganglion cell (RGC) function in mouse strains relevant to ... more To characterize differences in retinal ganglion cell (RGC) function in mouse strains relevant to disease models. C57BL/6J (B6) and DBA/2J (D2) are the two most common mouse strains; D2 has two mutated genes, tyrosinase-related protein 1 (Tyrp1) and glycoprotein non-metastatic melanoma protein B (Gpnmb), causing iris disease and intraocular pressure (IOP) elevation after 6 months of age that results in RGC degeneration, and is the most widely used model of glaucoma. DBA/2J.Gpnmb(+) (D2.Gpnmb(+)) is the wild type for the Gpnmb mutation and does not develop IOP elevation and glaucoma. Young (2-4 months of age) B6, D2, and D2.Gpnmb(+) mice (n=6 for each group) were tested with pattern electroretinogram (PERG) in response to different contrasts and spatial frequencies. PERG amplitude and latency dependencies on stimulus parameters (transfer functions) were established for each mouse strain, together with corresponding thresholds for contrast and spatial resolution. PERG analysis showed t...

Metabolic, pediatric, and systemic ophthalmology (New York, N.Y. : 1985), 1989
Experimental and clinical studies have demonstrated that the electroretinogram in response to per... more Experimental and clinical studies have demonstrated that the electroretinogram in response to periodical patterns alternating in contrast at constant mean luminance (pattern reversal ERG, P-ERG), is correlated with ganglion cell activity. Senile functional changes of these neurons might be therefore investigated by the P-ERG technique. Steady-state P-ERGs (8 Hz) in response to sinusoidal gratings of different spatial frequencies (0.6-6.8 c/d) were recorded in normal subjects ranging over 50 year age span. The P-ERG amplitude as a function of stimulus spatial frequency shows a maximum between 1.2-1.7 c/d and attenuation at higher and lower spatial frequencies (spatial tuning). The P-ERGs of the older subjects are reduced in amplitute, as compared to those of the younger ones, over the whole range of spatial frequency. This reduction is more marked at intermediate spatial frequencies resulting in a shallower tuning.

Vision research, 1992
We have recorded patterns electroretinograms (PERGs) and visual evoked potentials (VEPs) from 14 ... more We have recorded patterns electroretinograms (PERGs) and visual evoked potentials (VEPs) from 14 elderly subjects (mean age 72 yr) and 12 young subjects (mean age 21 yr) in response to stimulation by high contrast sinusoidal grating patterns of variable spatial frequency (at 9 Hz) and temporal frequency (at 1.7 c/deg). The major effect of aging on the PERG was an aspecific reduction in amplitude (of about 40%) at most spatial and temporal frequencies, together with a small but systematic phase lag. Control measurements suggest that senile miosis may be responsible for the phase lag, but not for the reduction in amplitude. The effects of aging on the VEP were more dramatic and depended on the spatial and temporal properties of the stimulus. VEP amplitudes (at 1.7 c/deg) were significantly lower for the aged at low temporal frequencies (below about 6 Hz), but were similar at high temporal frequencies. At 9 Hz, there was no effect of spatial frequency on VEP amplitude. At high temporal...

Investigative ophthalmology & visual science, 2014
We determined if the overlap of transient (tr) pattern electroretinograms (PERG(tr)) can explain ... more We determined if the overlap of transient (tr) pattern electroretinograms (PERG(tr)) can explain the generation of the steady-state (SS) pattern electroretinogram (PERG(SS)), and investigated the relationship between the two types of responses. Slightly jittered pattern reversals were used to generate quasi SS (QSS) PERG(SS) responses from eight normal subjects, recorded using lower eyelid skin electrodes, at rates between 6.9 and 26.5 reversals per second (rps). Jittered quasi PERG(SS) were deconvolved using the frequency domain continuous loop averaging deconvolution method. Additionally, conventional PERG(tr) at 2.2 rps and PERG(SS) at each of the QSS stimulation rates were obtained from all subjects. Two synthetic PERG(SS) responses were constructed at each stimulation rate, one using the PERG(tr) obtained at that rate, and the other using the conventional 2.2 rps PERG(tr). Synthetic responses then were compared to the recorded PERG(SS) using amplitude, latency, and spectral mea...
Nature neuroscience, 2000
Television and video games may be powerful triggers for visually induced epileptic seizures. To b... more Television and video games may be powerful triggers for visually induced epileptic seizures. To better understand the triggering elements of visual stimuli and cortical mechanisms of hyperexcitability, we examined eleven patients with idiopathic photosensitive epilepsy by recording visually evoked potentials (VEPs) in response to temporally modulated patterns of different contrast. For stimuli of low-medium, but not high, temporal frequency, the contrast dependence of VEP amplitude and latency is remarkably abnormal for luminance contrast (black-white), but not so for chromatic contrast (equiluminant red-green) stimuli. We conclude that cortical mechanisms of contrast gain control for pattern stimuli of relatively low temporal frequency and high luminance contrast are lacking or severely impaired in photosensitive subjects.
Human neurobiology, 1984
Pattern VEP measurements in infants below 2 months of age do not seem to show appreciable develop... more Pattern VEP measurements in infants below 2 months of age do not seem to show appreciable developmental changes in contrast sensitivity and cut-off spatial frequency in spite of the marked structural maturation that takes place in the visual system. This paper investigates the temporal parameters of the pattern VEP in order to establish electrophysiological criteria in detecting developmental changes of the visual pathway in this age range. In comparison with the adult, the neonatal response latency is delayed about 155 ms. The delay shortens during the first two months with a slope of around 1 ms/day. The neonatal temporal and spatial tuning functions, as compared with the adult ones, are markedly shifted towards lower temporal and spatial frequencies respectively, and do not show measurable changes in the early post-natal period.
Vision Research, 1995
LA CUESTA F.' e.

JAMA ophthalmology, 2014
IMPORTANCE We developed a novel strategy for treatment of Leber hereditary optic neuropathy (LHON... more IMPORTANCE We developed a novel strategy for treatment of Leber hereditary optic neuropathy (LHON) caused by a mutation in the nicotinamide adenine dinucleotide dehydrogenase subunit IV (ND4) mitochondrial gene. OBJECTIVE To demonstrate the safety and effects of the gene therapy vector to be used in a proposed gene therapy clinical trial. DESIGN AND SETTING In a series of laboratory experiments, we modified the mitochondrial ND4 subunit of complex I in the nuclear genetic code for import into mitochondria. The protein was targeted into the organelle by agency of a targeting sequence (allotopic expression). The gene was packaged into adeno-associated viral vectors and then vitreally injected into rodent, nonhuman primate, and ex vivo human eyes that underwent testing for expression and integration by immunohistochemical analysis and blue native polyacrylamide gel electrophoresis. During serial follow-up, the animal eyes underwent fundus photography, optical coherence tomography, and ...

Investigative ophthalmology & visual science, 2015
To address the permanent disability induced by mitochondrial dysfunction in experimental autoimmu... more To address the permanent disability induced by mitochondrial dysfunction in experimental autoimmune encephalomyelitis (EAE). Mice sensitized for EAE were rescued by intravitreal injection of adeno-associated viral vector serotype 2 with the complex I subunit gene scAAV-NDUFA6Flag. Controls were injected with a mitochondrially targeted red fluorescent protein (scAAV-COX8-cherry). Another group received scAAV-COX8-cherry, but was not sensitized for EAE. Serial pattern electroretinograms (PERGs) and optical coherent tomography (OCT) evaluated visual function and structure of the retina at 1, 3, and 6 months post injection (MPI). Treated mice were killed 6 MPI for histopathology. Immunodetection of cleaved caspase 3 gauged apoptosis. Complex I activity was assessed spectrophotometrically. Expression of NDUFA6Flag in the retina and optic nerve were evaluated between 1 week to 1 month post injection by RT-PCR, immunofluorescence and immunoblotting. Reverse transcription-PCR and immunoblot...
Uploads
Papers by Vittorio Porciatti