CHARACTERISTIC EXPRESSION OF CELL-ADHESION MOLECULES IN ADHALIN DEFICIENCY

We have reported the reduction of the B1 subunit of laminin and that o f heparan sulfate proteoglycan (HSPG) in two Japanese patients with ad halin deficiency. We here investigated immunohistochemically the expre ssion of cell adhesion molecules, including intercellular adhesion mol ecule-1 (ICAM-1), neural cell adhesion molecule (NCAM), and CD44 (HCAM ), in four Japanese patients with adhalin deficiency, compared to othe r types of muscular dystrophy. We found that NCAM was upregulated in a fair number of muscle fibers, regardless of the type of muscular dyst rophy. ICAM-1 was detected on the ran muscle cell membrane in all pati ents. CD44 was barely detected on the muscle cell membrane in adhalin deficiency, in contrast to the strong expression of CD44 which was obs erved in other types of muscular dystrophy. These findings suggest tha t a different degenerative or regenerative process is involved in adha lin deficiency compared to other types of muscular dystrophy.