Long-term evaluation of Leber's hereditary optic neuropathy-like symptoms in rotenone administered rats. (12th January 2015) - Zhang, Li
Highlights: Loss of visual function in rats after rotenone injection in the superior colliculus. Behavioral and electrophysiological data comparable to human clinical symptoms. Rotenone injection into rat superior colliculus serves as a model for LHON study. Abstract: Leber's hereditary optic neuropathy (LHON) is an inherited disorder affecting the retinal ganglion cells (RGCs) and their axons that lead to the loss of central vision. This study is aimed at evaluating the LHON symptoms in rats administered with rotenone microspheres into the superior colliculus (SC). Optical coherence tomography (OCT) analysis showed substantial loss of retinal nerve fiber layer (RNFL) thickness in rotenone injected rats. Optokinetic testing in rotenone treated rats showed decrease in head-tracking response. Electrophysiological mapping of the SC surface demonstrated attenuation of visually evoked responses; however, no changes were observed in the ERG data. The progressive pattern of disease manifestation in rotenone administered rats demonstrated several similarities with human disease symptoms. These rats with LHON-like symptoms can serve as a model for future investigators to design and implement reliable tests to assess the beneficial effects of therapeutic interventions for LHON disease.