Knockdown of Son a mouse homologue of the ZTTK syndrome gene causes neuronal migration defects and dendritic spine abnormalities

摘要

SON distribution in neural cells and in the developing mouse brain cortex. ( ) A schematic representation of the structure of full-length human and mouse SON. The arrow indicates the portion used as an antigen for antibody production. ( ) Characterization of the anti-SON antibody. E16.5 mouse brain lysates were used for Western blotting. The antibody detected multiple bands. The major band, the 260-kDa band indicated by the arrow, was almost completely absent after the antibody was absorbed with glutathione s-transferase-fusion antigen peptide (right lane). ( ) The localization of SON in the nucleus of Neuro-2a cells. Cells were stained with an anti-SON (green) or anti-SRSF2 (red) antibody. The nuclei were stained with DAPI (blue). The arrowheads indicate the colocalization of SON and SRSF2. ( ) Immunohistochemical distribution of SON in the developing mouse brain and its subcellular localization in mature neurons. The left panel shows a hematoxylin and eosin (HE)-stained section of the E15.5 mouse cerebral cortex. The layered structure of the developing cortex is shown on the left. MZ: marginal zone; CP: cortical plate; IZ: intermediate zone; SVZ: subventricular zone; VZ: ventricular zone. The middle panel shows the distribution of SON in an adjacent section to the HE-stained section. At E15.5, most neuronal progenitors and neurons expressed SON. The right upper panel shows a higher magnification view of the boxed area in the middle panel. SON was localized to the nucleus and was present as speckles, as in cultured cells. A similar subcellular distribution of SON was observed in mature neurons at P60 (the left lower panel)