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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.
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