CHANGES OF LAMININ BETA-2 CHAIN EXPRESSION IN CONGENITAL MUSCULAR-DYSTROPHY

We studied the distribution of laminin beta 2 chain in the skeletal mu scle basement membrane of 16 patients with congenital muscular dystrop hy (CMD) by immunohistochemistry. A dramatic reduction in the laminin beta 2 staining was observed in four patients with classical merosin-n egative CMD. A moderate reduction of laminin beta 2 labelling was obse rved in four patients with partial merosin deficiency and two patients with merosin-positive CMD. Two patients with merosin-positive CMD had no apparent changes in the expression of laminin beta 2. In three pat ients and one fetus diagnosed as Walker-Warburg syndrome (WWS) the lam inin beta 2 pattern was similar to normal controls. We conclude that a primary deficiency in the laminin alpha 2 chain may lead to a vast or moderate reduction in the laminin beta 2 chain in the skeletal muscle membrane. (C) 1997 Elsevier Science B.V.