Muscle histone deacetylase 4 upregulation in amyotrophic lateral sclerosis: potential role in reinnervation ability and disease progression.

@article{Bruneteau2013MuscleHD,
  title={Muscle histone deacetylase 4 upregulation in amyotrophic lateral sclerosis: potential role in reinnervation ability and disease progression.},
  author={G. Bruneteau and T. Simonet and S. Bauch{\'e} and Nathalie Mandjee and E. Malfatti and E. Girard and M. Tanguy and A. B{\'e}hin and F. Khiami and E. Sariali and Caroline Hell-Remy and F. Salachas and P. Pradat and E. Fournier and L. Lacomblez and J. Koenig and N. Romero and B. Fontaine and V. Meininger and L. Schaeffer and D. Hantaı̈},
  journal={Brain : a journal of neurology},
  year={2013},
  volume={136 Pt 8},
  pages={
          2359-68
        }
}
Amyotrophic lateral sclerosis is a typically rapidly progressive neurodegenerative disorder affecting motor neurons leading to progressive muscle paralysis and death, usually from respiratory failure, in 3-5 years. Some patients have slow disease progression and prolonged survival, but the underlying mechanisms remain poorly understood. Riluzole, the only approved treatment, only modestly prolongs survival and has no effect on muscle function. In the early phase of the disease, motor neuron… Expand
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References

SHOWING 1-10 OF 37 REFERENCES
Treatment with trichostatin A initiated after disease onset delays disease progression and increases survival in a mouse model of amyotrophic lateral sclerosis
TLDR
It is found that TSA ameliorated motoneuron death and axonal degeneration in SOD1-G93A mice and may provide a potential therapy to slow disease progression as well as to enhance motor performance to improve the quality of life for ALS patients. Expand
MicroRNA-206 Delays ALS Progression and Promotes Regeneration of Neuromuscular Synapses in Mice
TLDR
It is shown that a small noncoding RNA that is selectively expressed in skeletal muscle, miR-206, senses motor neuron injury or loss and helps ameliorate resultant muscle damage by promoting regeneration of neuromuscular synapses and slows disease progression in ALS. Expand
Muscle Gene Expression Is a Marker of Amyotrophic Lateral Sclerosis Severity
TLDR
Robust gene expression changes in skeletal muscle that can be of practical use when monitoring ALS status and the effects of disease-modifying drugs are provided. Expand
Selective vulnerability and pruning of phasic motoneuron axons in motoneuron disease alleviated by CNTF
TLDR
It is shown that in two mouse models of motoneuron disease, axons of fast-fatiguable motoneurons are affected synchronously, long before symptoms appear, and that mot oneuron disease involves predictable, selective vulnerability patterns by physiological subtypes of axons, episodes of abrupt pruning in the target region and compensation by resistant axons. Expand
A compensatory subpopulation of motor neurons in a mouse model of amyotrophic lateral sclerosis
TLDR
High‐resolution in vivo imaging in the G93A SOD1 mouse model showed that degenerative axon branches are easily distinguished from those undergoing compensatory reinnervation, showing fragmentation of terminal branches but sparing of the more proximal axon. Expand
Dose-ranging study of riluzole in amyotrophic lateral sclerosis
TLDR
A double-blind, placebo-controlled, multicentre study to confirm that riluzole is well tolerated and lengthens survival of patients with ALS and suggests that the 100 mg dose of rILuzole has the best benefit-to-risk ratio. Expand
Dose-ranging study of riluzole in amyotrophic lateral sclerosis. Amyotrophic Lateral Sclerosis/Riluzole Study Group II.
TLDR
A double-blind, placebo-controlled, multicentre study to confirm that riluzole is well tolerated and lengthens survival of patients with ALS, and suggests that the 100 mg dose of rILuzole has the best benefit-to-risk ratio. Expand
The deacetylase HDAC4 controls myocyte enhancing factor‐2‐dependent structural gene expression in response to neural activity
TLDR
It is shown that in skeletal muscle, HDAC4 is a critical modulator of MEF2‐dependent structural and contractile gene expression in response to neural activity, and suggests that activation ofHDAC4 in responded to chronically reduced neural activity suppresses MEF 2‐dependent gene expression and contributes to progressive muscle dysfunction observed in neuromuscular diseases. Expand
Early and Selective Loss of Neuromuscular Synapse Subtypes with Low Sprouting Competence in Motoneuron Diseases
TLDR
The results reveal pronounced subtype specificity in the anatomical plasticity and susceptibility to loss of neuromuscular synapses and suggest that degenerative motoneuron diseases involve a common early pathway of selective and progressive synaptic weakening also associated with aging. Expand
A Mutation Causes MuSK Reduced Sensitivity to Agrin and Congenital Myasthenia
TLDR
Immunocytochemical and electron microscopy studies of biopsied deltoid muscle showed dramatic changes in pre- and post-synaptic elements of the NMJs that induced a process of denervation/reinnervation in native NMJs and the formation, by an adaptive mechanism, of newly formed and ectopic NMJs. Expand
...
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3
4
...