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. 2015 Feb 18;35(7):3001–3009. doi: 10.1523/JNEUROSCI.3678-14.2015

Figure 4.

Figure 4.

Transgenic expression of Clcc1 rescues nm2453−/− phenotypes. A, Schematic of the RP24–306B18 BAC used to produce transgenic mice. B, The transgene (Tg-306B18) restores CLCC1 expression in nm2453−/− mice as shown by Western blot of cerebellar lysates from 1-month-old wild-type (+/+) or nm2453−/− (−/−) mice with (+) or without (−) the BAC transgene. GAPDH was used as a loading control. C–H, Transgenic expression of Clcc1 rescues cerebellar granule cell degeneration in nm2453−/− mice. Representative images of hematoxylin- and eosin-stained sagittal sections of cerebella from nm2453−/− and nm2453+/− (+/−) mice with or without the BAC transgene (Tg-306B18) at the indicated ages. Cerebellar lobules are indicated by Roman numerals. Higher-magnification images from lobule II are shown in G and H. Scale bars: F, 500 μm; H, 50 μm. I, Granule cells in lobule II of the cerebella from the indicated genotypes demonstrating that transgenic expression of Clcc1 reduces cell death in the nm2453−/− cerebellum. J, Axon degeneration was also rescued in nm2453−/− mice by transgenic expression of Clcc1, as shown by counts of nerve fibers in the femoral motor branch of mice with the same genotypes as in C–H. Values in I and J are means ± SEM (n = 3). *p < 0.01.