IOVS Journal of Applied Physiology
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(Investigative Ophthalmology and Visual Science. 2005;46:3420-3425.)
© 2005 by The Association for Research in Vision and Ophthalmology, Inc.
DOI:  10.1167/iovs.05-0038
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Impaired Neurite Outgrowth in the Retina of a Murine Model of Sandhoff Disease

Kazunori Sango,1,2 Masahiko Takano,2,3 Kyoko Ajiki,4 Akiko Tokashiki,1 Nobutaka Arai,5 Hitoshi Kawano,1 Hidenori Horie,6 and Shoji Yamanaka7

1From the Departments of Developmental Morphology, 4Basic Technics and Facilities, and 5Clinical Neuropathology, Tokyo Metropolitan Institute for Neuroscience, Tokyo, Japan; the 3Department of Ophthalmology, Kitasato University, School of Medicine, Sagamihara, Japan; the 6Advanced Research Center for Biological Science, Waseda University, Tokyo, Japan; and the 7Department of Pathology, Yokohama City University, School of Medicine, Yokohama, Japan.

PURPOSE. To investigate the effects of lysosomal storage on the morphologic appearance and the neurite outgrowth capability of the retina in a mouse model of GM2 gangliosidosis (Sandhoff disease).

METHODS. Histopathologic appearances of retinas in Sandhoff (SD) mice at 3 and 4 months of age were examined by light and electron microscopy. Retinas of SD mice and wild-type (WT) mice at 1, 2, and 4 months of age were cultured in collagen gel in the presence or absence of brain-derived neurotrophic factor (BDNF), and neurite outgrowth was examined.

RESULTS. Morphologic studies revealed accumulation of GM2 ganglioside in the retinal ganglion cells of SD mice in a time-dependent manner. The number of neurites from the retinal explants after 7 and 10 days in culture were significantly lower in 2- and 4-month-old SD mice than in the age-matched WT mice. The application of BDNF significantly improved neurite outgrowth from the retina in both SD and WT mice at 2 months of age. At 4 months of age, BDNF was much less effective at stimulating neurite outgrowth in the retina of SD mice than in retina of WT mice.

CONCLUSIONS. These results indicate that lysosomal storage of GM2 ganglioside impairs the capability of neurite outgrowth in retinal ganglion cells in culture and that BDNF is effective at diminishing this impairment during the early stage of the disease.






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