Skeletal muscle
- C9ORF72-related cellular pathology in skeletal myocytes derived from ALS-patient induced pluripotent stem cells
Summary: Evidence of protein aggregation and mitochondrial dysfunction were found in skeletal myocytes differentiated from ALS-patient induced pluripotent stem cells with the C9ORF72 mutation.
- Deterioration of mitochondrial bioenergetics and ultrastructure impairment in skeletal muscle of a transgenic minipig model in the early stages of Huntington's disease
Summary: Specific mitochondrial parameters in muscle of a minipig model of Huntington's disease can be used as potential biomarkers of disease progression and suggest that similar pathogenic mechanisms underlie disease progression in TgHD minipigs and HD patients.
- Therapeutic strategies for spinal muscular atrophy: SMN and beyond
Summary: Translational research for spinal muscular atrophy (SMA) should address the development of non-CNS and survival motor neuron (SMN)-independent therapeutic approaches to complement and enhance the benefits of CNS-directed and SMN-dependent therapies.
- Poor maternal nutrition and accelerated postnatal growth induces an accelerated aging phenotype and oxidative stress in skeletal muscle of male rats
Summary: Muscle of ‘developmentally programmed’ rat offspring demonstrated accelerated aging and oxidative stress, which could explain why some individuals are at greater risk of developing age-associated muscular dysfunction than others.
- Angiotensin-(1-7) attenuates disuse skeletal muscle atrophy in mice via its receptor, Mas
Summary: In this article, the authors demonstrate that a peptide with actions mainly in the cardiovascular system prevents the skeletal muscle damage induced by disuse.
- The role of mTOR signaling in the regulation of protein synthesis and muscle mass during immobilization in mice
Summary: The activation of mTOR signaling is both necessary and sufficient to alleviate the decreases in protein synthesis and muscle mass that occur during immobilization.
- Phospholamban overexpression in mice causes a centronuclear myopathy-like phenotype
Summary: Phospholamban overexpression in mouse slow-twitch muscle impairs SERCA function and causes histopathological features associated with human centronuclear myopathy.