Is there a maturation defect related to calcium in muscle mitochondria from dystrophic mice and Duchenne and Becker muscular dystrophy patients?

Lucas-Heron, Brigitte; Mussini, Jean‐Marie; Ollivier, Béatrice

doi:10.1016/0022-510x(89)90116-0

Cited by 22 publications

(5 citation statements)

References 15 publications

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“…MEF2A expression by central nuclei may also contribute to establishing the nature of mdx muscle plasticity during regeneration, given the regulatory role of MEF2 in myogenesis, hypertrophy, activity, or stress responses (McKinsey et al, 2002a, b). It is interesting that atypical proliferation and defects in differentiation have been reported in cultures of muscle cells isolated from patients with Duchenne or congenital muscular dystrophy (Miike, 1983; Jasmin et al, 1984; Lucas‐Heron et al, 1989, 1994; Oexle and Kohlschutter, 2001).…”

Section: Discussionmentioning

confidence: 99%

Satellite cells from dystrophic (Mdx) mice display accelerated differentiation in primary cultures and in isolated myofibers

Yablonka–Reuveni

Anderson

2005

Developmental Dynamics

109

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In the dystrophic (mdx) mouse, skeletal muscle undergoes cycles of degeneration and regeneration, and myogenic progenitors (satellite cells) show ongoing proliferation and differentiation at a time when counterpart cells in normal healthy muscle enter quiescence. However, it remains unclear whether this enhanced satellite cell activity is triggered solely by the muscle environment or is also governed by factors inherent in satellite cells. To obtain a better picture of myogenesis in dystrophic muscle, a direct cell-by-cell analysis was performed to compare satellite cell dynamics from mdx and normal (C57Bl/10) mice in two cell culture models. In one model, the kinetics of satellite cell differentiation was quantified in primary cell cultures from diaphragm and limb muscles by immunodetection of MyoD, myogenin, and MEF2. In mdx cell cultures, myogenin protein was expressed earlier than normal and was followed more rapidly by dual myogenin/MEF2A expression and myotube formation. In the second model, the dynamics of satellite cell myogenesis were investigated in cultured myofibers isolated from flexor digitorum brevis (FDB) muscle, which retain satellite cells in the native position. Consistent with primary cultures, satellite cells in mdx myofibers displayed earlier myogenin expression, as well as an enhanced number of myogenin-expressing satellite cells per myofiber compared to normal. The addition of fibroblast growth factor 2 (FGF2) led to an increase in the number of satellite cells expressing myogenin in normal and mdx myofibers. However, the extent of the FGF effect was more robust in mdx myofibers. Notably, many myonuclei in mdx myofibers were centralized, evidence of segmental regeneration; all central nuclei and many peripheral nuclei in mdx myofibers were positive for MEF2A. Results indicated that myogenic cells in dystrophic muscle display accelerated differentiation. Furthermore, the study demonstrated that FDB myofibers are an excellent model of the in vivo state of muscle, as they accurately represented the dystrophic phenotype.

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Section: Discussionmentioning

confidence: 99%

Satellite cells from dystrophic (Mdx) mice display accelerated differentiation in primary cultures and in isolated myofibers

Yablonka–Reuveni

Anderson

2005

Developmental Dynamics

109

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“…A recent study linked the DAP in muscle stem cells undergoing asymmetric division with the epigenetic activation [10]. Furthermore, myogenic cells from both Duchenne patients [11,12] and mdx mice [13] show abnormalities of a range of important functions including altered proliferation and differentiation and defects of energy metabolism with disorganized mitochondrial networks [14]. Moreover, a purinergic phenotype involving an increased expression and activity of P2XR7 receptors has been identified in myoblasts of the mdx mouse model of Duchenne type muscular dystrophy [15,16] as well as in human DMD lymphoblasts [17].…”

Section: Introductionmentioning

confidence: 99%

Dystrophic mdx mouse myoblasts exhibit elevated ATP/UTP-evoked metabotropic purinergic responses and alterations in calcium signalling

Róg

Oksiejuk

Gosselin

et al. 2019

Biochimica et Biophysica Acta (BBA) - Molecular Basis of Diseas

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Running title: mdx mutation affects calcium signalling in myoblastsHighlights:1. NGS reveals changes in Ca 2+ signalling-related RNAs in mdx myoblasts 2. Mdx myoblasts exerts increased susceptibility to P2RY2-mediated stimulation 3. Levels of several Ca 2+ signalling-related proteins are changed in mdx myoblasts 4. P2RY2 agonist slows-down mdx myoblasts motility SummaryPathophysiology of Duchenne Muscular Dystrophy is still elusive. Although progressive damage to muscle fibres is a cause of muscle deterioration leading to premature death, there is a growing body of evidence indicating that the triggering effects of DMD mutation are present at the very early stage of muscle development. Previously, elevated activity of P2X7 receptors and increased store-operated calcium entry were shown in myoblasts derived from mdx mice. Here, the metabotropic extracellular ATP/UTP-evoked response has been investigated. Sensitivity to antagonist, effect of gene silencing and cellular localization studies showed that elevated purinergic responses in mdx myoblasts have been caused by increased expression of P2Y2 but not P2Y4 receptors. These alterations have physiological implications as shown by reduced motility of mdx myoblasts upon treatment with P2Y2 agonist. The ultimate increase in intracellular calcium in dystrophic cells reflected complex alterations of calcium homeostasis identified in the RNA seq data and with significant modulation at the protein level including a decrease of Gq11 subunit , PMCA, IP3-receptor and elevation of PLC, SERCA and NCX. In conclusion, whereas specificity of dystrophic myoblast excitation by extracellular ATP is determined by specific receptor overexpression, the intensity of this altered response depends on relative activities of downstream calcium regulators that also accompany the Dmd gene mutation. These results confirm that phenotypic effects of DMD emerge in undifferentiated muscle cells and not only due to the absence of dystrophin protein in myofibres. Therefore, the pathogenesis of DMD and the relevance of current therapeutic approaches may need re-evaluation.

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“…Parvalbumin and the mitochondria1 calcium-binding protein calmitine. are reduced in the skeletal muscles of several rodent mutants, including dystrophic mice 5,9,10,[13][14][15]221. Whether the decrease of these calciumbinding proteins reflects an immature state of the muscle fibres.…”

Section: Introductionmentioning

confidence: 99%

Parvalbumin immunohistochemistry in denervated skeletal muscle

Olivé

Ferrer

1994

Neuropathology Appl Neurobio

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Parvalbumin is a calcium-binding protein which, in muscle, is mainly found in type 2B fibres, whereas type 1 fibres lack parvalbumin immunoreactivity. Previous studies have shown that this pattern is highly dependent upon motor neuron innervation and is modified in denervated, cross-reinnervated or chronic low-frequency stimulated muscles. In the present study, we have examined the modifications of parvalbumin immunocytochemistry in the anterior tibialis muscle of the rat at different intervals following section of the sciatic nerve. During the first 2 weeks after denervation, no changes in parvalbumin immunoreactivity were seen, although a global reduction of fibre diameter was observed. Three weeks after denervation, small angulated, strongly parvalbumin-immunoreactive fibres appeared. From the second month onwards, the pattern of parvalbumin immunohistochemistry was characterized by areas composed of small, strongly immunoreactive fibres separated by less atrophic areas displaying a normal chequerboard distribution of parvalbumin immunoreactivity. The increase of parvalbumin-immunoreactivity in denervated and reinnervated muscle, as seen in our study, indicates that important changes in parvalbumin distribution occurs in muscle fibres after denervation. These changes are probably produced in an attempt to bind the free cytosolic calcium which accumulates in denervated fibres, and further reinforces the role of parvalbumin in calcium homeostasis during denervation and reinnervation.

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Is there a maturation defect related to calcium in muscle mitochondria from dystrophic mice and Duchenne and Becker muscular dystrophy patients?

Cited by 22 publications

References 15 publications

Satellite cells from dystrophic (Mdx) mice display accelerated differentiation in primary cultures and in isolated myofibers

Satellite cells from dystrophic (Mdx) mice display accelerated differentiation in primary cultures and in isolated myofibers

Dystrophic mdx mouse myoblasts exhibit elevated ATP/UTP-evoked metabotropic purinergic responses and alterations in calcium signalling

Parvalbumin immunohistochemistry in denervated skeletal muscle

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