We have undertaken an immunohistochemical study of
laminin subunits in the central nervous system (CNS) of fetuses and patients with
Fukuyama congenital muscular dystrophy (FCMD) and of controls including five fetuses. Immunoreaction product deposits with
antibodies to
laminin alpha 1, alpha 2, beta 1 and gamma 1, and
beta-dystroglycan were detected on the surface and vessels of the CNS of controls. No staining with anti-
alpha-sarcoglycan antibody was detected in the CNS. Neurons and glia did not react with any of the
antibodies used. In utero expression of
laminin subunits and
beta-dystroglycan seemed to be lower in the cerebrum than in the spinal cord. Moreover, immunostaining for
laminin alpha 2 and beta 1 tended to be weak on the fetal spinal cord surface. Expression of
laminin subunits and
dystrophin-associated proteins in the CNS may be modulated during development, as in the skeletal muscle. The distribution of immunoreaction product deposits was basically the same in FCMD and controls, although
laminin alpha 2 and
beta-dystroglycan expression appeared to be decreased in the CNS of the FCMD cases. Defects of the pial-glial barrier of the fetal brain surface have been considered the main cause of
micropolygyria in FCMD, and these observations suggest that the co-localization and secondary loss of these
proteins in association with the unknown product(s) of the FCMD gene might be involved in the CNS lesions of this disorder.