LAPBBS4 can rescue motile cilia defects and hydrocephalus.

<p>A) SEM image of cilia from the lateral ventricles of the brain in 6 months old WT, <i>Bbs4^−/−</i>and <i>BBS4^tg</i> mice. Motile cilia appear normal and healthy in <i>BBS4^tg</i> mice. Right panel shows the motile cilia in higher magnification. <b>B)</b><i>Bbs4^−/−</i> mice have hydrocephalus (enlarged ventricles, arrowheads). The top panel shows an MRI of 3-month-old mouse brains in coronal section, and the bottom panel shows the horizontal sections of the same mice. The ventricles are slightly visible in the WT mice and in the <i>Bbs4^tg</i> mice. <b>C)</b> Relative volume of lateral ventricle is significantly low (***P<0.001) in WT and <i>BBS4^tg</i> mice compared to the <i>Bbs4^−/−</i>mice (data shown as mean +/− SEM, n = 3 per genotype).