LAMININ BETA-2 CHAIN AND ADHALIN DEFICIENCY IN THE SKELETAL-MUSCLE OFWALKER-WARBURG SYNDROME (CEREBRO-OCULAR DYSPLASIA-MUSCULAR DYSTROPHY)

Muscular dystrophy may be caused by disturbances in a number, of muscl e proteins that appear to be part of a chain of interacting molecules that includes cytoskeletal, cell membrane, and basement membrane compo nents. We found that the skeletal muscle cells in two cases of Walker- Warburg syndrome were severely deficient in the laminin beta 2 chain a nd in adhalin. The findings indicate that these two proteins are key m olecules in the interactive protein complex conferring muscle stabilit y and cell survival.