Changes of laminin β2 chain expression in congenital muscular dystrophy

Ronald D. Cohn, Ralf Herrmann, Ulla M. Wewer, Thomas Voit

Research output: Contribution to journalArticlepeer-review

Abstract

We studied the distribution of laminin β2 chain in the skeletal muscle basement membrane of 16 patients with congenital muscular dystrophy (CMD) by immunohistochemistry. A dramatic reduction in the laminin β2 staining was observed in four patients with classical merosin-negative CMD. A moderate reduction of laminin β2 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 β2. In three patients and one fetus diagnosed as Walker-Warburg syndrome (WWS) the laminin β2 pattern was similar to normal controls. We conclude that a primary deficiency in the laminin α2 chain may lead to a vast or moderate reduction in the laminin β2 chain in the skeletal muscle membrane.

Original languageEnglish (US)
Pages (from-to)373-378
Number of pages6
JournalNeuromuscular Disorders
Volume7
Issue number6-7
DOIs
StatePublished - Sep 1997

Keywords

  • Congenital muscular dystrophy
  • Extracellular matrix
  • Immunohistochemistry
  • Laminin
  • Walker-Warburg syndrome

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Neurology
  • Clinical Neurology
  • Genetics(clinical)

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