Infantile nystagmus syndrome (INS); Optokinetic response (OKR); Visual pathway abnormality; Ocular motor system; Vestibulo-ocular reflex (VOR); Albinism; Congenital nystagmus (CN); Zebrafish
Chen Chien-Cheng, Huang Melody Ying-Yu, Weber Konrad P., Straumann Dominik, Bockisch Christopher J. (2014), Afternystagmus in darkness after suppression of optokinetic nystagmus: an ocular motor response to a motion aftereffect driven by retinal afterimages, in Experimental Brain Research
Huber-Reggi Sabina P., Mueller Kaspar P., Straumann Dominik, Huang Melody Ying-Yu, Neuhauss Stephan C. F. (2014), Individual Larvae of the Zebrafish Mutant belladonna Display Multiple Infantile Nystagmus-Like Waveforms that Are Influenced by Viewing Conditions, in Investigative Ophthalmology & Visual Science
, 55(6), 3971-3978.
Chen Chien-Cheng, Bockisch Christopher J., Olasagasti Itsaso, Weber Konrad P., Straumann Dominik, Huang Melody Ying-Yu (2014), Positive or Negative Feedback of Optokinetic Signals: Degree of the Misrouted Optic Flow Determines System Dynamics of Human Ocular Motor Behavior, in Investigative Ophthalmology & Visual Science
, 55(4), 2297-2306.
Chen Chien-Cheng, Bockisch Christopher, Bertolini Giovanni, Olasagasti Itsaso, Neuhauss Stephan, Weber Konrad, Straumann Dominik, Huang Melody Ying-Yu (2014), Velocity storage mechanism in zebrafish larvae, in The Journal of Physiology
, 592(1), 203-214.
Traber Ghislaine L, Chen Chien-Cheng, Huang Ying-Yu, Spoor Marcella, Roos Jeanine, Frens Maarten A, Straumann Dominik, Grimm Christian (2012), Albino mice as an animal model for infantile nystagmus syndrome., in Investigative ophthalmology & visual science
, 53(9), 5737-47.
Huber-Reggi Sabina P, Chen Chien-Cheng, Grimm Lea, Straumann Dominik, Neuhauss Stephan C F, Huang Melody Ying-Yu (2012), Severity of infantile nystagmus syndrome-like ocular motor phenotype is linked to the extent of the underlying optic nerve projection defect in zebrafish belladonna mutant., in The Journal of neuroscience : the official journal of the Society for Neuroscience
, 32(50), 18079-86.
To date, the pathomechanism of infantile nystagmus syndrome (INS), an ocular motor disorder characterized by involuntary horizontal eye oscillations, is unknown. My discovery of achiasmatic zebrafish belladonna (bel) mutant with an INS-like behavioral phenotype promises new insights into the etiology of INS. Just like in achiasmatic zebrafish bel mutant, visual pathway abnormalities are commonly present in INS patients and may, therefore, be a factor in the pathogenesis of INS. To substantiate this hypothesis, I will measure the ocular motor behavior of normals and subjects with INS when exposed to an artificially generated inverted retinal slip. To understand the role of the vestibulo-ocular reflex (VOR) in INS, I will examine the development of the VOR in achiasmatic zebrafish bel mutant. I will use achiasmatic zebrafish bel mutant to evaluate and discover new pharmacological treatments for INS. Finally, I plan to develop a new diagnostic method for visual pathway abnormalities.