Properties of acetylcholine-receptor activation in human Duchenne muscular dystrophy myotubes

Mancinelli, E.; Sardini, Alessandro; D'Aumiller, A.; Meola, Giovanni; Martucci, Giuseppe; Cossu, Giulio; Wanke, Enzo

doi:10.1098/rspb.1989.0047

Cited by 5 publications

(1 citation statement)

References 41 publications

(50 reference statements)

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“…However it has been reported more recently that DMD myotubes express the dystrophin deficiency [66]. Patch-clamp studies of activation properties of the nicotinic acetylcholine receptor in DMD myotubes also revealed that the average frequency of channel openings was about four times higher in DMD myotubes [25]. Patch-clamp studies of activation properties of the nicotinic acetylcholine receptor in DMD myotubes also revealed that the average frequency of channel openings was about four times higher in DMD myotubes [25].…”

Section: D) Duchenne Muscular Dystrophy (Dmd)mentioning

confidence: 97%

Hereditary human myopathies in muscle culture

Meola

1991

Ital J Neuro Sci

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In this article I illustrated the use of regenerating human muscle cultures for studying the hereditary human myopathies. Although some of the data are still controversial, they do point up the great potential of this "in vitro system". For hereditary myopathies due to developmentally regulated proteins that are expressed only at a more advanced stage of muscle differentiation, the use of highly differentiated nerve-muscle cocultures might contribute significantly to a better understanding of their developmental pathogenesis. More advanced techniques (permanent human muscle cell lines, heterokaryons, myoblast transfer, gene transfer, myogenic conversion of human non-muscle cells, cybrid clones) may provide a great deal of information at molecular level and may also have practical applications in the diagnosis or even in the treatment of hereditary human myopathies.

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Section: D) Duchenne Muscular Dystrophy (Dmd)mentioning

confidence: 97%

Hereditary human myopathies in muscle culture

Meola

1991

Ital J Neuro Sci

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Amphiphilic properties of molecular forms of acetylcholinesterase in normal and dystrophic muscle

Cabezas‐Herrera

Campoy

Vidal

1994

J of Neuroscience Research

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Acetylcholinesterase (AChE) molecular forms were studied in normal (NM) and in dystrophic (DM) 129B6F1/J mouse muscle. Successive extractions of the tissue with saline and saline-Triton X-100 buffers yielded two soluble fractions, S1 and S2. Forty percent of the AChE in NM was measured in S1 and 60% in S2, and 65% and 35%, respectively, in extracts from DM. A12, A8, G4, G2, and G1 forms of AChE were found in S1 and S2 from NM and DM. A similar content of asymmetric molecules was noticed between NM and DM. G4 AChE was a minor species in DM, and G1 and G2 AChE were more abundant in DM than in NM. The amphiphilic properties of the several molecules were assessed by Triton X-114 phase-partitioning and hydrophobic chromatography. Thirty and 70% of the enzyme in a mixture of S1 and S2 partitioned in the detergent-rich and in the detergent-poor phases, respectively, whether the extracts were obtained from NM or DM. Asymmetric and G4 AChE predominated in the aqueous phase and G1 and G2 in the detergent phase. Ten and 25% of the enzyme in S1 from NM or DM, respectively, was adsorbed to the phenyl-agarose. Elution of the retained enzyme followed by sedimentation analysis revealed that a certain amount of asymmetric and most of the G1 and G2 forms were associated with the matrix. The content of amphiphilic asymmetric and light globular forms was notably higher in DM than in NM. The results suggest that dystrophic muscle produces a specific pattern of molecular forms of AChE.

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Electrocardiographic findings in mdx mice: A cardiac phenotype of Duchenne muscular dystrophy

Chu

Otero²,

Lopez³

et al. 2002

Muscle and Nerve

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The mdx mouse is a model of Duchenne muscular dystrophy (DMD). As many DMD patients die of cardiac failure, we investigated whether mdx mice exhibited clinically relevant cardiac phenotypes. We applied a recently developed method for noninvasively recording electrocardiograms (ECGs) to study male mdx mice (n = 15) and control mice (n = 15). The mdx mice had significant tachycardia and decreased heart rate variability, consistent with observations in DMD patients. Heart rate was nearly 15% faster in mdx mice than control mice (P < 0.05). The rate-corrected QT interval duration and PR interval were shorter in mdx compared to control mice (P < 0.05). The muscarinic antagonist atropine significantly increased heart rate and decreased PR interval in C57 mice. In contrast, atropine significantly decreased heart rate and increased PR interval in all mdx mice. Pharmacological autonomic blockade and baroreflex sensitivity testing demonstrated an imbalance in autonomic nervous system modulation of heart rate, with decreased parasympathetic activity and increased sympathetic activity in mdx mice. Baseline ECGs and contrary responses to muscarinic blockade by atropine in mice deficient in neuronal nitric oxide synthase (nNOS) suggest that the autonomic dysfunction in mdx mice may be independent of decreased myocardial nNOS. These electrocardiographic findings in dystrophin-deficient mice may provide new bases for diagnosing, understanding, and treating DMD patients.

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Properties of acetylcholine-receptor activation in human Duchenne muscular dystrophy myotubes

Cited by 5 publications

References 41 publications

Hereditary human myopathies in muscle culture

Hereditary human myopathies in muscle culture

Amphiphilic properties of molecular forms of acetylcholinesterase in normal and dystrophic muscle

Electrocardiographic findings in mdx mice: A cardiac phenotype of Duchenne muscular dystrophy

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