Abnormal neuromuscular junctions in the lateral rectus muscle of wobbler mice.
We have studied the lateral rectus muscles and neuromuscular junctions (NMJs) of abducens motoneurons in wobbler (wr/wr) mutant mice from 26 to 58 days of age. The muscles of wr/wr weighed about 70% of the weight of littermate controls and were composed of fiber types comparable to those of controls, as assayed by succinate dehydrogenase activity. The most obvious difference between wr/wr and control NMJs was a reduction in the length of the postjunctional membrane of wr/wr mice. The mutant muscle endplate membrane was only about 70% (6.58 micron) the length of control muscle regions (9.44 micron). There were no obvious differences at the light microscopic level in the distribution of acetylcholine (ACh) receptors at junctional regions or staining of acetylcholinesterase, as assayed with alpha-bungarotoxin binding or enzyme histochemistry. Indirect immunocytochemical studies using antibodies directed against the subunits of the ACh receptor failed to indicate an abnormal presence of immature receptors clustered at the NMJs of wr/wr mice. Our findings suggest that the formation or maintenance of normal postjunctional folds and the differentiation of receptors at the junctions are under independent control during development. Furthermore, the wobbler mutation may affect muscle cell differentiation as well as neuronal differentiation. This mutant mouse should prove a useful model for study of postjunctional fold formation and function.
LaVail
J H
JH
Neuroscience Program, University of California, San Francisco 94143.
Irons
K P
KP
D000375:Aging / Q000378:metabolism*; D000818:Animals; D002452:Cell Count; D006651:Histocytochemistry; D051379:Mice; D008810:Mice, Inbred C57BL; D008818:Mice, Neurologic Mutants / Q000033:anatomy & histology* / Q000254:growth & development; D008854:Microscopy, Electron; D024510:Muscle Development; D009132:Muscles / Q000378:metabolism / Q000648:ultrastructure*; D009469:Neuromuscular Junction / Q000378:metabolism / Q000648:ultrastructure*; D010641:Phenotype; D011950:Receptors, Cholinergic / Q000378:metabolism*
D011950:Receptors, Cholinergic