Sarcoplasmic–endoplasmic–reticulum Ca2+‐ATPase and calsequestrin are overexpressed in spared intrinsic laryngeal muscles of dystrophin‐deficient mdx mice

@article{Ferretti2009SarcoplasmicendoplasmicreticulumC,
  title={Sarcoplasmic–endoplasmic–reticulum Ca2+‐ATPase and calsequestrin are overexpressed in spared intrinsic laryngeal muscles of dystrophin‐deficient mdx mice},
  author={R. Ferretti and M. Marques and A. Pertille and H. Santo Neto},
  journal={Muscle \& Nerve},
  year={2009},
  volume={39}
}
In the mdx mouse model of Duchenne muscular dystrophy, the lack of dystrophin is associated with increased calcium levels and skeletal muscle myonecrosis. The intrinsic laryngeal muscles (ILM) are protected and do not undergo myonecrosis. We investigated whether this protection is related to an increased expression of calcium‐binding proteins, which may protect against the elevated calcium levels seen in dystrophic fibers. The expression of sarcoplasmic–endoplasmic–reticulum Ca2+‐ATPase and… Expand
Increased sarcolipin expression and decreased sarco(endo)plasmic reticulum Ca2+ uptake in skeletal muscles of mouse models of Duchenne muscular dystrophy
TLDR
The data suggest that sarcolipin upregulation is a common secondary alteration in all dystrophic muscles and contributes to the abnormal elevation of intracellular Ca2+ concentration via SERCA inhibition. Expand
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TLDR
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TLDR
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TLDR
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Mitigation of muscular dystrophy in mice by SERCA overexpression in skeletal muscle.
TLDR
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Recent advances in the understanding of calcium ion cycling through the sarcolemma, the sarcoplasmic reticulum and mitochondria, and its involvement in the pathogenesis of muscular dystrophies are summarized. Expand
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TLDR
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Overexpression of SERCA1a in the mdx diaphragm reduces susceptibility to contraction-induced damage.
Although the precise pathophysiological mechanism of muscle damage in dystrophin-deficient muscle remains disputed, calcium appears to be a critical mediator of the dystrophic process. DuchenneExpand
Isobaric Tagging-Based Quantification for Proteomic Analysis: A Comparative Study of Spared and Affected Muscles from mdx Mice at the Early Phase of Dystrophy
TLDR
Overall, the shotgun technique proved to be suitable to perform quantitative comparisons between distinct dystrophic muscles and allowed the suggestion of new potential biomarkers and drug targets for dystrophinopaties. Expand
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References

SHOWING 1-10 OF 54 REFERENCES
Subproteomics analysis of Ca2+‐binding proteins demonstrates decreased calsequestrin expression in dystrophic mouse skeletal muscle
TLDR
The reduced 2D 'Stains-All' pattern of luminal Ca(2+)-binding proteins in mdx preparations supports the calcium hypothesis of muscular dystrophy. Expand
Drastic reduction of sarcalumenin in Dp427 (dystrophin of 427 kDa)-deficient fibres indicates that abnormal calcium handling plays a key role in muscular dystrophy.
TLDR
It is shown that a key luminal Ca2+-binding protein SAR (sarcalumenin) is affected in mdx skeletal-muscle fibres, and this confirms the idea that abnormalCa2+ cycling is involved in Ca2-induced myonecrosis. Expand
Subproteomics analysis of Ca+-binding proteins demonstrates decreased calsequestrin expression in dystrophic mouse skeletal muscle.
TLDR
The reduced 2D 'Stains-All' pattern of luminal Ca(2+)-binding proteins in mdx preparations supports the calcium hypothesis of muscular dystrophy. Expand
Drastic reduction of calsequestrin-like proteins and impaired calcium binding in dystrophic mdx muscle.
Although the reduction in dystrophin-associated glycoproteins is the primary pathophysiological consequence of the deficiency in dystrophin, little is known about the secondary abnormalities leadingExpand
Comparative analysis of Dp427-deficient mdx tissues shows that the milder dystrophic phenotype of extraocular and toe muscle fibres is associated with a persistent expression of beta-dystroglycan.
TLDR
This study suggests that the rescue of dystrophin-associated glycoproteins, and possibly the increased removal of cytosolic Ca2+ ions, might also play an important role in protecting muscle cells from necrotic changes. Expand
Reduced expression of regucalcin in young and aged mdx diaphragm indicates abnormal cytosolic calcium handling in dystrophin-deficient muscle.
TLDR
The results from the proteomics analysis of dystrophic diaphragm support the concept that abnormal Ca2+ -handling is involved in x-linked muscular dystrophy. Expand
The expression of the neonatal sarcoplasmic reticulum Ca2+ pump (SERCA1b) hints to a role in muscle growth and development.
TLDR
It is suggested that the insufficient function and development of the diaphragm in the SERCA1 null mutant mice may be due to the lack ofSERCA1b, and an important regulation of SER CA1b expression at the protein level is pointed to. Expand
Sarcoplasmic reticulum function in slow- and fast-twitch skeletal muscles from mdx mice
TLDR
The results indicate that mdx hindlimb muscles are affected differently by the disease process and suggest that a reduced ability of the Ca2-ATPase to load Ca2+ and a leaky sarcoplasmic reticulum membrane may be involved in the altered intracellular Ca 2+ homeostasis. Expand
Targeted Disruption of the ATP2A1 Gene Encoding the Sarco(endo)plasmic Reticulum Ca2+ ATPase Isoform 1 (SERCA1) Impairs Diaphragm Function and Is Lethal in Neonatal Mice*
TLDR
The absence ofSERCA1 in type II fibers, and the absence of compensatory increases in other Ca2+ handling proteins, coupled with the marked increase in contractile function required of the diaphragm muscle to support postnatal respiration, can account for respiratory failure in term SERCA1-null mice. Expand
Increased calcium influx in dystrophic muscle
TLDR
The results suggest that increased calcium influx, as a result of increased leak channel activity, could result in the elevated [Ca2+]i in dystrophic muscle. Expand
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