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. 2014 May 10;2:54. doi: 10.1186/2051-5960-2-54

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© Groh et al.; licensee BioMed Central Ltd. 2014

This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

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Loss of retinal ganglion cells and axonal perturbation in optic nerves of Cln3 ^-/- mice. A: Cresyl violet staining of retinal flat mount preparations and quantification of Nissl + retinal ganglion cells in 18-month-old Cln3 ^+/+ and Cln3 ^-/- mice (n = 4 per group). Scale bar: 50 μm. B: NeuN and C: Brn3a immunohistochemistry on retinal sections from 18-month-old Cln3 ^+/+ and Cln3 ^-/- mice. Scale bar, 30 μm. A loss of NeuN + or Brn3a + retinal ganglion cells in Cln3-/- mice was obvious. D: Representative electron micrograph of optic nerve cross-sections from 18-month-old Cln3-/- mice. Axonal spheroids with accumulation of organelles and dense bodies were frequently observed. E: Immunohistochemistry using antibodies against non-phosphorylated neurofilaments (SMI32; brownish precipitate) in longitudinal optic nerve sections of 18-month-old Cln3 ^-/- mice visualizes axonal spheroids. Scale bar: 30 μm. F: Quantification revealed that SMI32+ axonal spheroids are barely detectable in optic nerves of Cln3 ^+/+ mice but accumulate in 18-month-old Cln3 ^-/- mice (n = 4 per group). Student’s t-test. ***P < 0.001.