Secondary storage of GM2 ganglioside and cholesterol in Fuca1-deficient mice. Secondary storage of GM2 ganglioside and cholesterol in Fuca1-deficient mice.

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Secondary storage of GM2 ganglioside and cholesterol in Fuca1-deficient mice. Secondary storage of GM2 ganglioside and cholesterol in Fuca1-deficient mice. (A) GM2 immunoreactivity is prominent in the CNS of 3-month-old Fuca1-deficient (−/−) mice but not in wild-type mice (WT). Magnified images of the boxed areas (∼2.5×) are shown on the right. Scale bars: 25 µm. (B) Accumulation of free cholesterol was shown by filipin staining, particularly in the molecular layer (ML) but not in the granular layer (GL) of the cerebellum of knockout mice at the age of 3 and 11 months. By contrast, WT mice were devoid of such large cholesterol deposits. Magnified images of the boxed areas (∼2.5×) are shown on the right. Scale bars: 50 µm. Heike Wolf et al. Dis. Model. Mech. 2016;9:1015-1028 © 2016. Published by The Company of Biologists Ltd