2016
DOI: 10.1016/j.neuint.2015.12.013
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Changes in structure and function of diaphragm neuromuscular junctions from BACHD mouse model for Huntington's disease

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Cited by 15 publications
(11 citation statements)
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“…Skeletal muscle atrophies, and neuromuscular junction function becomes progressively abnormal in the R6/2 mouse model of HD (Ribchester et al, 2004). Dysfunction of neuromuscular junctions has also been reported in the diaphragm of another rodent model of HD, the BACHD mouse (de Aragão et al, 2016). These findings suggest that muscle pathologies, in addition to loss of descending control of motor function, are likely to contribute to the motor deficits seen in HD mice.…”
Section: Introductionmentioning
confidence: 73%
“…Skeletal muscle atrophies, and neuromuscular junction function becomes progressively abnormal in the R6/2 mouse model of HD (Ribchester et al, 2004). Dysfunction of neuromuscular junctions has also been reported in the diaphragm of another rodent model of HD, the BACHD mouse (de Aragão et al, 2016). These findings suggest that muscle pathologies, in addition to loss of descending control of motor function, are likely to contribute to the motor deficits seen in HD mice.…”
Section: Introductionmentioning
confidence: 73%
“…Contradicting the denervation idea is that a morphological analysis of R6/2 mouse neuromuscular junctions showed defects in only a very small fraction of neuromuscular junctions (Ribchester et al, 2004). A more recent study has also reported morphological changes, including vesicle shape at the neuromuscular junction of R6/2 mice, but showed no clear evidence of denervation (de Aragão et al, 2016). Moreover, re-innervation after crushing the motor neuron was normal in R6/2 skeletal muscle (Ribchester et al, 2004).…”
Section: Denervationmentioning
confidence: 99%
“…It is also possible that postsynaptic receptors are altered, as suggested by a recent study showing that acetylcholine receptors (measured by α ‐bungarotoxin binding) are reduced in the diaphragm of BACHD mice (de Aragão et al. ). However, earlier work examined cardiac β 1‐adrenergic receptor densities in R6/2 and WT mice but did not see any genotypic differences (Mihm et al.…”
Section: Discussionmentioning
confidence: 99%