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Changes of laminin beta 2 chain expression in congenital muscular dystrophy

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We studied the distribution of laminin beta 2 chain in the skeletal muscle basement membrane of 16 patients with congenital muscular dystrophy (CMD) by immunohistochemistry. A dramatic reduction in the laminin beta 2 staining was observed in four patients with classical merosin-negative CMD. A moderate reduction of laminin beta 2 labelling was observed 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 patients and one fetus diagnosed as Walker-Warburg syndrome (WWS) the laminin 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.
OriginalsprogEngelsk
TidsskriftNeuromuscular Disorders
Vol/bind7
Udgave nummer6-7
Sider (fra-til)373-8
Antal sider6
ISSN0960-8966
StatusUdgivet - 1 sep. 1997

ID: 34325896