Figure 1.

A) Western blotting using an antibody against glycosylated α-DG (VIA4-1). Bands around 120 kDa are seen in fetal and adult controls, but there are no bands in FCMD patients. B) RT-PCR using the same cases as those in A). DG mRNA is slightly expressed in controls, and increased in FCMD cases, especially in cerebrum. C) Immunohistochemistry using antibodies against glycosylated α-DG (IIH6C4), core-α-DG (α-DG-p), and N^ε-(carboxymethyl)lysine (CML). In fetal and post-natal FCMD cases, glycosylated α-DG is reduced in the glia limitans, but core-α-DG is maintained (a, b, d, e). CML accumulates slightly in astrocytes of FCMD cases compared to controls (c, f). Cortical neurons and neuropils are negative for glycosylated α-DG both in FCMD cases and controls, but positive for core α-DG (d, e, g, h, j, k). Capillaries are positive for both glycosylated and core α-DG (arrows). CML accumulates slightly in neurons of FCMD cases compared to controls (i, l). Arrowheads indicate glia limitans.

y: years old, w: gestational weeks, Cr: cerebrum, Sp: spinal cord, Cont: control