Dystrophin and its isoforms in a sympathetic ganglion of normal and dystrophic mdx mice: immunolocalization by electron microscopy and biochemical characterization.
In normal mouse superior cervical ganglion, dystrophin immunoreactivity is present in ganglionic neurons, satellite cells and Schwann cells. It is associated with several cytoplasmic organelles and specialized plasma membrane domains, including two types of structurally and functionally different intercellular junctions: synapses, where it is located at postsynaptic densities, and adherens junctions. Dystrophin immunostaining can be ascribed to the 427,000 mol. wt full-length dystrophin, as well as to the several dystrophin isoforms present in superior cervical ganglion, as revealed by western immunoblots. In mdx mouse superior cervical ganglion, which lacks the 427,000 mol. wt dystrophin, the unchanged pattern of dystrophin immunolabelling observed at several subcellular structures indicates the presence of dystrophin isoforms at these sites. Moreover, the absence of labelled adherens junctions indicates the presence of full-length dystrophin at this type of junction in the normal mouse superior cervical ganglion. The lower number of immunopositive postsynaptic densities in mdx mouse superior cervical ganglion than in normal mouse ganglion suggests the presence, in the latter, of postsynaptic densities with differently organized dystrophin cytoskeleton: some containing dystrophin isoforms alone or together with 427,000 mol. wt dystrophin, and others containing 427,000 mol. wt dystrophin alone.
De Stefano
M E
ME
Dipartimento di Biologia Cellulare e dello Sviluppo, Università La Sapienza, Roma, Italy.
Zaccaria
M L
ML
Cavaldesi
M
M
Petrucci
T C
TC
Medori
R
R
Paggi
P
P
D000818:Animals; D016189:Dystrophin / Q000378:metabolism*; D004591:Electrophoresis, Polyacrylamide Gel; D005728:Ganglia, Sympathetic / Q000378:metabolism* / Q000473:pathology; D015151:Immunoblotting; D007536:Isomerism; D051379:Mice; D008810:Mice, Inbred C57BL; D018101:Mice, Inbred mdx; D008854:Microscopy, Electron; D008970:Molecular Weight; D009137:Muscular Dystrophy, Animal / Q000378:metabolism* / Q000473:pathology; D017783:Superior Cervical Ganglion / Q000378:metabolism / Q000473:pathology
D016189:Dystrophin