A5072596127- Retinal Development and Disorders
- Vestibular and auditory disorders
- Photoreceptor and optogenetics research
- Neuroscience and Neuropharmacology Research
- Connexins and lens biology
- Ophthalmology and Visual Impairment Studies
- Ophthalmology and Eye Disorders
- Glaucoma and retinal disorders
- Hearing, Cochlea, Tinnitus, Genetics
- Neural dynamics and brain function
- Sleep and Wakefulness Research
- Circadian rhythm and melatonin
- Retinopathy of Prematurity Studies
- Ion channel regulation and function
- Retinal Diseases and Treatments
- Neuroscience of respiration and sleep
- Ocular and Laser Science Research
- Visual perception and processing mechanisms
- Corneal surgery and disorders
- Ion Channels and Receptors
- Aortic Disease and Treatment Approaches
- Down syndrome and intellectual disability research
- Optical Coherence Tomography Applications
- Insect and Arachnid Ecology and Behavior
- Fetal and Pediatric Neurological Disorders
Erasmus MC
2004-2023
Netherlands Institute for Neuroscience
2013-2023
Royal Netherlands Academy of Arts and Sciences
2013-2023
Erasmus University Rotterdam
2004-2023
New York University
2002-2014
Objective Disrupting thalamocortical activity patterns has proven to be a promising approach stop generalized spike‐and‐wave discharges (GSWDs) characteristic of absence seizures. Here, we investigated what extent modulation neuronal firing in cerebellar nuclei (CN), which are anatomically an advantageous position disrupt cortical oscillations through their innervation wide variety thalamic nuclei, is effective controlling Methods Two unrelated mouse models seizures were used: the natural...
To measure contrast sensitivity in C57BL/6, the most commonly used mouse behavioral neuroscience, and to study effect of sex, age, miotic drugs on function. In addition, authors tested a mutant which plasticity cerebellum is impaired by expressing protein kinase C inhibitor. This inhibitor also expressed retina, possibly affecting vision.The gain optokinetic reflex (OKR) decreases as stimuli become more difficult see. Recording OKR gains evoked moving sine gratings shows whether stimulus was...
Thoracic aortic aneurysm and dissection (TAAD) are associated with connective tissue disorders like Marfan syndrome Loeys-Dietz syndrome, caused by mutations in the fibrillin-1, TGFβ-receptor 1- -2 genes, SMAD3 TGFβ2 but have also been ascribed to ACTA2 gene adults, spread throughout gene. We report on a novel de novo c.535C>T exon 6 leading p.R179C aminoacid substitution toddler girl primary pulmonary hypertension, persistent ductus arteriosus, extensive cerebral white matter lesions, fixed...
Abstract Myopia is the most common developmental disorder of juvenile eyes, and it has become an increasing cause severe visual impairment. The GJD2 locus been consistently associated with myopia in multiple independent genome-wide association studies. However, despite strong genetic evidence, little known about functional role refractive error development. Here, we find that depletion gjd2a (Cx35.5) or gjd2b (Cx35.1) orthologs zebrafish, changes biometry status eye. Our immunohistological...
Purpose.: To study three-dimensional optokinetic eye movements of wild-type C57BL/6J mice, the most commonly used mouse in oculomotor physiology. Optokinetic are reflexive that use visual feedback to minimize image motion across retina. These gaze-stabilizing reflexes a prominent model system for studying motor control and learning. They three dimensional consist horizontal, vertical, torsional component. Methods.: Eye were evoked by sinusoidally rotating virtual sphere equally spaced dots...
Congenital nystagmus, involuntary oscillating small eye movements, is commonly thought to originate from aberrant interactions between brainstem nuclei and foveal cortical pathways. Here, we investigated whether nystagmus associated with congenital stationary night blindness (CSNB) results primary deficits in the retina. We found that CSNB patients as well an animal model (nob mice), both of which lacked functional nyctalopin protein (NYX, nyx) ON bipolar cells (BCs) at their synapse...
The cerebellum is divided into multiple parasagittally organized modules, which are thought to represent functional entities. How individual modules participate in cerebellar control of complex movements such as locomotion remains largely unknown. To a large extent, this caused by the inability study contribution during locomotion. Because architecture based on narrow, elongated cortical strips that may be discontinuous rostrocaudal direction, lesion complete module, without affecting...
The climbing fibers (CFs) that project from the dorsal cap of inferior olive (IO) to flocculus cerebellar cortex have been reported be purely sensory, encoding "retinal slip." However, a clear oculomotor projection nucleus prepositus hypoglossi (NPH) IO has shown. We therefore studied sensorimotor information is present in CF signal. presented rabbits with visual motion noise stimuli break up tight relation between instantaneous retinal slip and eye movement. Strikingly, about motor behavior...
Humans with Down syndrome (DS) and Ts65Dn mice both show a reduced volume of the cerebellum due to significant reduction in density granule neurons. Recently, cerebellar hypoplasia was rescued by single treatment SAG, an agonist Sonic hedgehog pathway, administered on day birth. In addition normalizing morphology, this restored ability learn spatial navigation task, which is associated hippocampal function. It not clear what extent improved performance results from restoration architecture...
In addition to the well-known signals of retinal image slip, floccular complex spikes (CSs) also convey nonvisual signals. We recorded eye movement and CS activity from Purkinje cells in awake rabbits sinusoidally oscillated dark on a vestibular turntable. The stimulus frequency ranged 0.2 1.2 Hz, velocity amplitude 6.3 50°/s. average modulation was evaluated at each combination amplitude. More than 75% carried this remained relatively constant over entire range. phase response opposite that...
A bstract : Modulation of the complex spike activity Purkinje cells in cerebellar flocculus can convey not only visual signals but also nonvisual signals. The modulation, which is readily observed with vestibular stimulation awake rabbit darkness, approximately in‐phase concomitant simple modulation. This nonreciprocal relationship contrasts to reciprocal found when afforded vision.
Abstract Purpose To study the effectiveness of high‐dose atropine for reducing eye growth in Mendelian myopia children and mice. Methods We studied effect with progressive without a monogenetic cause. Children were matched age axial length (AL) their first year treatment. considered annual AL progression rate as outcome compared rates percentile charts an untreated general population. treated C57BL/6J mice featuring myopic phenotype Donnai–Barrow syndrome by selective inactivation Lrp2 knock...
Recently, a Y727C variant in the dual-specific 3′,5′-cyclic nucleotide phosphodiesterase 11A (PDE11A-Y727C) was linked to increased sleep quality and reduced myopia risk humans. Given well-established role that PDE11 substrates cAMP cGMP play eye physiology sleep, we determined if (1) PDE11A protein is expressed retina or other segments mice, (2) PDE11A-Y7272C affects catalytic activity and/or subcellular compartmentalization more so than nearby suicide-associated PDE11A-M878V variant, (3)...
Congenital nystagmus is a condition where the eyes of patients oscillate, mostly horizontally, with frequency between 2 and 10 Hz. Historically, believed to be caused by maladaptation oculomotor system thus considered disease brain stem. However, we have recently shown that congenital associated stationary night blindness synchronously oscillating retinal ganglion cells. In this perspective article, discuss how some details can accounted for mechanism propose.
Abstract In Nyx nob mice, a model for congenital nystagmus associated with stationary night blindness (CSNB), synchronous oscillating retinal ganglion cells (RGCs) lead to oscillatory eye movements, i.e. nystagmus. Given the specific expression of mGluR6 and Ca v 1.4 in photoreceptor bipolar cell synapses, as well their clinical association CSNB, we hypothesize that Grm6 nob3 1.4‐KO mutants show, like mouse, oscillations both RGC activity movements. Using multi‐electrode array recordings...
Abstract In Nyx nob mice, a model for congenital nystagmus associated with stationary night blindness (CSNB), synchronous oscillating retinal ganglion cells (RGCs) lead to oscillatory eye movements, i.e., nystagmus. Given the distribution of mGluR6 and Cav1 . 4 in retina as well their clinical association CSNB, we hypothesize that -/- mutants show, like mouse, oscillations originate A II amacrine (A ACs). Using movement multi-electrode array (MEA) recordings RGCs show underlying RGC are also...
Abstract Congenital nystagmus, involuntary oscillating small eye movements, is commonly thought to originate from aberrant interactions between brainstem nuclei and foveal cortical pathways. Here we investigated whether nystagmus associated with congenital stationary nightblindness (CSNB) can result primary deficits in the retina. We found that CSNB patients as well an animal model ( nob mice), both of which lack functional nyctalopin protein (NYX, nyx) ON bipolar cells (ON-BC) at their...