Figure 2.

The molecular pathomechanism leading to brain anomaly in Fukuyama-type congenital MD (FCMD). (A) On the normal cerebral surface, α-dystroglycan in the glia limitans binds to laminin in the basal lamina. (B) Glycosylation of α-dystroglycan is defective in FCMD, which causes disruption of the dystroglycan-laminin binding, leading to misassembly of laminin and disorganization of basal lamina. This facilitates the overmigration of neuronal cells through the fragmented basal lamina to the subarachnoid space, and results in disarray of cerebral cortical layering and malformation of gyri.