Immunofluorescence microscopy of a myopathy: α-Actinin is a major constituent of nemaline rods

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 antibodie...

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Veröffentlicht in:Experimental cell research 1980-01, Vol.127 (2), p.409-420
Hauptverfasser: Jockusch, Brigitte M., Veldman, Henk, Griffiths, Gareth W., Van Oost, Bernard A., Jennekens, Frans G.I.
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Sprache:eng
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Zusammenfassung: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.
ISSN:0014-4827
1090-2422
DOI:10.1016/0014-4827(80)90445-0