PMID: 9160897Apr 1, 1997Paper

A comparative freeze-fracture study of plasma membrane of dystrophic skeletal muscles in dy/dy mice with merosin (laminin 2) deficiency and mdx mice with dystrophin deficiency

Neuropathology and Applied Neurobiology
Seiji ShibuyaI Nonaka

Abstract

The intramembranous particle (IMP), orthogonal array (OA) and orthogonal array subunit particle (OASP) densities of skeletal muscle plasma membranes of merosin deficient dy/dy mice and their control mice at 7, 14 and 28 days after birth were analysed by freeze-fracture electron microscopy. Similar studies were performed on dystrophin-deficient mdx mice with mild muscle weakness at 28 days after birth for the comparison with those of dy/dy mice with severe muscle weakness at the same age. In the pre-clinical stage of dy/dy mice at 14 days after birth, the membranes showed a significantly decreased density of OAs (P<0.01 by Wilcoxon rank-sum test) as compared with control mice, while those in the clinical stage of dy/dy mice at 28 days after birth showed normal IMP density but a marked depletion of OA density (P<0.01). Moreover, at 28 days after birth, the reduction of OAs in the plasma membranes of dy/dy mice was more marked than that of mdx mice (P<0.05 by Wilcoxon rank-sum test). These results provided us with the information that the OA density was affected more severely with merosin deficiency than with dystrophin deficiency, and again supported our previously proposed concept that the clinical severity in muscular dystrophi...Continue Reading

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Citations

Mar 27, 2010·Journal of Biomedicine & Biotechnology·Yoshihiro Wakayama
Jan 31, 2006·Micron : the International Research and Review Journal for Microscopy·Koutarou HayashiHiroaki Oniki
Jul 28, 2016·Acta Histochemica Et Cytochemica·Minenori Ishido, Tomohiro Nakamura
Aug 29, 2007·Muscle & Nerve·Maria Julia MarquesHumberto Santo Neto

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