title: Long-term evaluation of Leber's hereditary optic neuropathy-like symptoms in rotenone administered rats.
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.
authors.affiliation: Eye Center, Second Affiliated Hospital, Medical School of Zhejiang University, Hangzhou, China; Ophthalmology, Keck School of Medicine, University of Southern California, Los Angeles, CA,USA.
authors.forename: Li
authors.initials: L
authors.lastname: Zhang
authors.affiliation: Department of Ophthalmology, Chang Gung Memorial Hospital, Linkou Medical Center, Taoyuan, Taiwan.
authors.forename: Laura
authors.initials: L
authors.lastname: Liu
authors.affiliation: Ophthalmology, Keck School of Medicine, University of Southern California, Los Angeles, CA,USA.
authors.forename: Ann L
authors.initials: AL
authors.lastname: Philip
authors.affiliation: Ophthalmology, Keck School of Medicine, University of Southern California, Los Angeles, CA,USA.
authors.forename: Juan C
authors.initials: JC
authors.lastname: Martinez
authors.affiliation: Ophthalmology, Keck School of Medicine, University of Southern California, Los Angeles, CA,USA.
authors.forename: Juan C
authors.initials: JC
authors.lastname: Guttierez
authors.affiliation: Molecular and Experimental Medicine, The Scripps Research Institute, La Jolla, CA,USA.
authors.forename: Mathieu
authors.initials: M
authors.lastname: Marella
authors.affiliation: Molecular and Experimental Medicine, The Scripps Research Institute, La Jolla, CA,USA.
authors.forename: Gaurav
authors.initials: G
authors.lastname: Patki
authors.affiliation: Molecular and Experimental Medicine, The Scripps Research Institute, La Jolla, CA,USA.
authors.forename: Akemi
authors.initials: A
authors.lastname: Matsuno-Yagi
authors.affiliation: Molecular and Experimental Medicine, The Scripps Research Institute, La Jolla, CA,USA.
authors.forename: Takao
authors.initials: T
authors.lastname: Yagi
authors.affiliation: Ophthalmology, Keck School of Medicine, University of Southern California, Los Angeles, CA,USA. Electronic address: biju.thomas@med.usc.edu.
authors.forename: Biju B
authors.initials: BB
authors.lastname: Thomas
mesh_terms: D000818:Animals; D004195:Disease Models, Animal; D004596:Electroretinography; D005074:Evoked Potentials, Visual; D006801:Humans; D008863:Microspheres; D029242:Optic Atrophy, Hereditary, Leber / Q000139:chemically induced / Q000503:physiopathology*; D051381:Rats; D012402:Rotenone; D013477:Superior Colliculi / Q000503:physiopathology; D041623:Tomography, Optical Coherence
chemical_list: D012402:Rotenone
keywords: Animal model; Leber’; Rotenone; Superior colliculus; Visual function; s hereditary optic neuropathy