Immunofluorescence microscopy of a myopathy : α-Actinin is a major constituent of nemaline rods |
| |
Authors: | Brigitte M Jockusch Henk Veldman Gareth W Griffiths Bernard A Van Oost Frans G I Jennekens |
| |
Institution: | 1. European Molecular Biology Laboratory, Heidelberg, West Germany;2. Department of Neurology University Hospital, Utrecht, The Netherlands;3. Department of Hematology, University Hospital, Utrecht, The Netherlands |
| |
Abstract: | A biopsy of skeletal muscle taken from a child with the clinical symptoms of congenital nemaline myopathy was studied. Light and electron microscopy revealed rod-like structures within the muscle fibres, and thus confirmed the clinical diagnosis. Indirect immunofluorescence, using specific antibodies against actin and desmin (both derived from chicken gizzard) as well as against α-actinin and tropomyosin (both from porcine skeletal muscle) revealed that the rods consist of massive accumulations of α-actinin. Desmin seems to be peripherally associated with the rods. Anti-actin and anti-tropomyosin did not stain the rods; however, a masking effect could not be ruled out. These findings support previous hypotheses that nemaline rods can be taken to be lateral-polymers of normal Z-disks. |
| |
Keywords: | |
本文献已被 ScienceDirect 等数据库收录! |
|