Fig. 7.

AxD patient and human control immunohistochemistry analysis of FABP7 and UGT8.A and B, the demographics of the samples analyzed are as follows: AxD patient #1 is a 3-year-old female with an R239H GFAP mutation, and AxD patient #2 is an 8-year-old male with an R416W GFAP mutation, and the control is a 4-year-old female with no neuropathology. A, anti-FABP7 staining. Ai, normal subcortical white matter from a control with no neuropathology. The antibody labels cells with several fine, branching processes, indicative of astrocytes. The arrow points to one astrocyte. The normal appearing white matter in the AxD patients is similar (data not shown). Aii, in this image of AxD white matter from AxD patient #1, the normal structures have been replaced by astrocytes and RFs, one of the largest is pointed out (arrow). The scale bars are 100 μm. B, anti-UGT8 staining. Bi, normal subcortical white matter from a control with no neuropathology. The antibody labels the cytoplasm of small cells with round nuclei, indicative of oligodendrocytes (arrows point to two oligodendrocytes). Just above the bottom oligodendrocyte is a blood vessel, which is negative. Bii, in this image of subpial area of the cerebral cortex of patient #2, where RFs accumulate, astrocytes contain punctate, positive signal in the cell body (black arrows). Round to oval profiles represent RFs (red arrow), many of which stain peripherally, a common feature of RF immunostaining. The astrocyte at the top right is multinucleated, a common feature of AxD astrocytes and is shown at higher magnification in the inset. Biii, in this section of AxD subcortical white matter from AxD patient #1, showing an astrocyte (black arrow), positive staining of astrocyte end feet around a blood vessel (short arrow), RFs (one shown with red arrow), and an oligodendrocyte (green arrow). In the normal appearing white matter in the AxD sections, the antibody stained only oligodendrocytes, as in controls (data not shown). Scale bars are 50 μm.