Normal development of muscle fibers and motor end plates in dystrophic mice

1978 ◽  
Vol 58 (3) ◽  
pp. 570-574 ◽  
Author(s):  
Thomas G. Burch ◽  
Peter K. Law
1977 ◽  
Vol 40 (4) ◽  
pp. 836-843 ◽  
Author(s):  
S. Carbonetto

1. Neuromuscular transmission was studied in the extensor digitorum-longus muscle of dystrophic mice (strain 129/ReJ) by means of intracellular recording techniques. 2. In a large population of normal and dystrophic muscle fibers tested, the incidence of transmission failure was about 2% and showed no significant difference between the two groups. 3. Quantal size and quantum content of dystrophic junctions were found to be normal. This was true even of nerve terminal on apparently atrophied muscle fibers. 4. The facilitation ratio at dystrophic junctions was not significantly different from normal. 5. Dystrophic neuromuscular junctions exhibited an abnormality high frequency of giant spontaneous potentials. Application of tetrodotoxin (10(-6) M) and curare (10(-6) M) indicated that these potentials were caused by impulse-independent release of acetylcholine. 6. Neuromuscular transmission in dystrophic mice was found functionally normal and unrelated to the degenerative state of the muscle.


2020 ◽  
Vol 34 (S1) ◽  
pp. 1-1
Author(s):  
Thomas A Kwiatkowski ◽  
Aubrey Rose ◽  
Rachel Jung ◽  
Kevin McElhanon ◽  
Brian Paleo ◽  
...  

1997 ◽  
Vol 272 (1) ◽  
pp. C333-C340 ◽  
Author(s):  
A. De Luca ◽  
S. Pierno ◽  
D. C. Camerino

The membrane electrical properties of diaphragm and extensor digitorum longus (EDL) muscle fibers of dystrophic mdx and control mice from 4 wk to 14-19 mo of age were recorded with the intracellular microelectrode technique. Up to 8 wk of age, the diaphragm and EDL muscles did not differ between the two strains. From 8 up to 20 wk, the mdx diaphragm fibers showed a higher membrane resistance (Rm), which was due to significantly lower values of resting chloride conductance (GCl) and an overexcitability with respect to age-matched controls. Oppositely, the mdx EDL muscle fibers had significantly lower Rm and higher GCl values than age-related controls at 8, 10, and 13 wk, along with a decreased membrane excitability. These differences were no longer detectable at 20 wk. The diaphragm and EDL muscles from 14- to 19-mo-old controls showed a decrease of GCl and an increase of potassium conductance with respect to adult animals. In aged mdx animals, these changes were very dramatic in diaphragm fibers, whereas no differences, with respect to adults, were found in the EDL muscle. Thus GCl is an index of the dystrophic condition of mdx muscles. In the degenerating diaphragm, the impairment of GCl can account for some of the pathological features of the muscle. In the EDL muscle, the changes of GCl can follow the high regenerative potential of the hindlimb muscles of the mdx phenotype.


1983 ◽  
Vol 6 (1) ◽  
pp. 3-13 ◽  
Author(s):  
Lynne M. Kerr ◽  
Nick Sperelakis

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